Exam 3

Question 1

What is the most common collagen vascular or connective tissue disease in the US?

Answer 1

Lupus Erythematous

Question 2

Answer 2

Cementoblastoma

Question 3

What is the most common cutaneous lymphoma?

Answer 3

Mycosis Fungoides

Question 4

Answer 4

Erythema Multiforme (Minor)

Question 5

What is the other name for Mucous Membrane Pemphigoid?

Answer 5

Cicatricial Pemphigoid (twice as common as pemphigus)

Question 6

Periapical Cemento-Osseous Dysplasia lesions start out as completely radiopaque. True or False?

Answer 6

False, they start out as Radiolucent and become mixed and then end as radioopaque with a radiolucent rim.

Question 7

Answer 7

Tuberous Sclerosis

• System complex characterized by:
  
  • CNS manifestations
  • Mental retardation
  • Seizure disorders – some medications can cause gingival overgrowth
  • Potato-like growths (“tubers”)
  • Angiofibromas of the skin
  • Ungual (or periungual) fibromas
  • Characteristic skin lesions
    
    • Shagreen patches
    • Ash-leaf spots (this is a hypo-pigmentation, and it was on his boards, shagreen patches and café-ale are hyperpigmentation)
• Facial angiofibromas:
  
  • Multiple, smooth-surfaced papules occurring primarily on the nasolabial fold area
• Ungual fibromas:
  
  • Similar to angiofibromas clinically
  • Seen around or under the margins of the nails
• Shagreen patches
  
  • Connective tissue hamartomas
• Ash-leaf spots
  
  • Ovoid areas of hypopigmentation
• Tubers in 85%, seizure disorder in 75%, mental retardation in 40%
• Rare tumor of the heart muscle, termed cardiac rhabdomyoma, is also associated in this syndrome
• Angiomyolipomas are found on the kidney
• Oral manifestations:
  
  • Developmental enamel pitting on the facial aspect of the anterior permanent teeth
  • Multiple fibrous papules
• Treatment of this condition is directed toward management of the seizure disorder & periodic imaging studies
• Patients have a reduced life span – death is usually via CNS or kidney disease

Question 8

Where are the four most common lcoations of Langerhans Cell Histiocytosis?

Answer 8

Bone presentation

1. Skull
2. Ribs
3. Verebrae
4. Mandible

Question 9

Answer 9

Fibrous Dysplasia - Monostotic

• Accounts for 80% of all cases
• Jaws are among the most commonly affected sites
• Usually diagnosed in teenage years
• M=F
• Painless, slowly-growing swelling of the affected area is the most common feature
• Maxilla > Mandible – he won’t ask us this
• XRAY:
• Chief radiographic feature is a fine “ground-glass” opacification - like a shower door
  
  • Results from superimposition of poorly calcified bone in a disorganized fashion
• Lesions are not well demarcated radiographically – the margins blend imperceptibly into the adjacent normal bone
• PA radiographs may show narrowing of PDL with an ill-defined lamina dura
• Involvement of the mandible leads to expansion of both buccal and lingual plates
• Maxilla involvement leads to obliteration of the maxillary sinus

Question 10

Vitamin B6 name and function?

Answer 10

Vitamin B6 (pyridoxine): cofactor associated with enzymes that participate in amino acid synthesis.

Question 11

What is the most common form of cancer involving bone?

Answer 11

Metastatic Carcinoma

Question 12

What is CREST syndrome?

Answer 12

Don’t need to know clinical picture for this.

A system complex characterized by:

C – Calcinosis cutis

R – Raynaud’s phenomenon – also associated with systemic sclerosis

E – Esophageal dysfunction

S – Sclerodactyly

T – Telangiectasia

• Most patients affected are women in their 60s
• Calcinosis cutis:
  
  • Multiple, movable, nontender, subcutaneous, nodular calcifications
• Raynaud’s phenomenon:
  
  • Observed when hands or feet are exposed to cold temperatures
  • Initial sign is a dramatic blanching; digits appear white as a result of severe vasospasm
  • A few minutes later, the affected areas turn blue due to venous stasis
  • After warming, the digits turn red with the return of hyperemic blood flow; accompanied by varying degrees of throbbing pain
• Esophageal dysfunction:
  
  • Caused by abnormal collagen deposition in the esophageal submucosa
• Sclerodactyly
  
  • Fingers become stiff
  • Skin becomes smooth & shiny
  • Fingers undergo permanent flexure, resulting in a characteristic “claw” deformity
  • Resembles the hands of systemic sclerosis and like systemic sclerosis, the change is due to abnormal deposition of collagen within the dermis
• Telangiectasias:
  
  • Numerous scattered red papules, 1-2 mm in size
  • Blanch (indicates the red color is due to blood contained within blood vessels)
  • In this case, blood is contained in small collections of dilated capillaries termed telangiectasias that are close to the surface of the mucosa
  • Most frequently found on the vermilion zone of lips & facial skin
  • Significant bleeding may occur
• Treatment is the same as that of systemic sclerosis; however, CREST syndrome is not as severe

Patients should be monitored for pulmonary hypertension; if it occurs, it usually will 10 or more years after initial diagnosis

Question 13

What is Hailey-Hailey Disease?

Answer 13

Familial Pemphigus, and has test-tube like ridges.

Question 14

Answer 14

Ash leaf spot

Question 15

Answer 15

Peripheral Giant Cell Granuloma

• Reactive lesion caused by local irritation or trauma – Usually a bluish hint
• Occurs exclusively on the gingiva or edentulous alveolar ridge – Unlike Pyogenic Granuloma
• Appears as an erythematous mass similar to PG, although PGCG is often more blue or purple compared to the bright red PG
• Average age is 35, 60% in females
• May produce a “cupping” resorption of the underlying alveolar bone
• Treatment is local surgical excision down to underlying bone
• Adjacent teeth should be scaled
• 10% recur

Question 16

What is an odontogenic cyst?

Answer 16

It is a pathologic cavity lined by odontogenic epithelium and filled with fluid or semisolid material. All comes from dental lamina. And all of these tumors and cysts originate from Rests of Serres mostly, and some rests of Malassez.

Question 17

What are the four most commonly affected bones of Paget’s Disease?

Answer 17

1. Vertebrae
2. Pelvis
3. Skull
4. Femur

Question 18

What is the most widely used treatment for ameloblastomas?

Answer 18

Marginal Resection, recurrence rate is 15%. Some surgeons take 1.5 cm margins beyond what is visible radiographically.

Question 19

Answer 19

Glandular Odontogenic Cyst

• Developmental cyst with glandular and/or salivary gland features
• The most recently named Odontogenic Cyst
• Second most common to cross midline, next to central giant cell granuloma, if a tooth is involved, assume GOC rather than CGCG
• Avg age = 50
• 75% of cases occur in the mandible
  
  • Predilection for anterior jaws – know this
• Size can vary from small to very large
• XRAY:
  
  • Either unilocular or multilocular radiolucency
  • Well-defined margins
  • Sclerotic rim
• Treatment is enucleation or curettage
  
  • 30% will recur – The other one with 30% recurrence rate is odontogenic keratocyst
• Multilocular lesions are more likely to recur than unilocular lesions
• Therefore, en bloc resection is used at times

Question 20

What is the first part of treatment regardless of the patient having symptoms for mucous membrane pemphigoid?

Answer 20

Refer to an opthalmologist

Question 21

What is Batson’s plexus?

Answer 21

• This is a valveless vertebral venous plexus that might allow retrograde spread of tumor cells & bypass the lungs.
• And is one possible explanation of H&N metastases in the absence of lung metastases

Question 22

What are the characteristics of Pernicious Anemia?

Answer 22

• Megaloblastic anemia
• Caused by poor absorption of cobalamin (Vitamin B12, extrinsic factor)
• Most patients lack intrinsic factor because of autoimmune destruction of the parietal cells of the stomach, which results in decreased absorption of cobalamin.
• Oral manifestations: atrophic glossitis (This tongue is also red and bald, but this one is beefier and balder)

Question 23

What are the characteristics of maligant bone neoplasia?

Answer 23

• Usually symptomatic
• Grows more rapidly
• Invades and destroys adjacent structures (cortex) – teeth aren’t being displaced so the teeth are floating, not a good sign, and usually indicative of malignancy rather than benign
• Often asymmetrical
• Ragged or poorly defined margins and destroys cortex
• Laying down bone outside the cortex
• Capable of metastasis

Question 24

In what part of the mouth do ameloblastomas appear 85% of the time?

Answer 24

Mandible, molar-ascending ramus area

Question 25

Vitamin B2 name and function?

Answer 25

Vitamin B2 (riboflavin): necessary for cellular oxidation-reduction reactions. Oral alterations like angular cheilitis, glossitis.

Question 26

What is secondary amyloidosis associated with?

Answer 26

Hemodialysis

Question 27

Answer 27

Ameloblastoma

“Resorption of Roots is Common”

• “Soap bubble” – when the RL loculations are large
• “Honeycombed” – when the loculations are small
• **Buccal and lingual cortical expansion is frequently present**

Question 28

What syndrome are Odontogenic Keratocysts associated with?

Answer 28

Nevoid Basal Cell Carcinoma syndrome (Gorlin Syndrome)

Question 29

What pathology has a “blood-soaked sponge” appearance?

Answer 29

Aneurysmal bone cyst

Question 30

Which pathology is a odontogenic neoplasm of cementoblasts?

Answer 30

Cementoblastoma

Question 31

What dermatologic disease is often seen with GI cancer?

Answer 31

Acanthosis Nigricans. It can be seen in conjunction with gastrointestinal cancer: - similar to internal malignancy associated with Lesser Trelat syndrome (associated with multiple cancers, not just GI).

Question 32

Answer 32

Ectodermal Dysplasia

• Group of inherited conditions in which two or more ectodermal derived anatomic strictures fail to develop
  
  • Skin, hair, nails, teeth, sweat glands
• Over 170 types
• Best known is hypohidrotic ectodermal dysplasia
  
  • X-linked; male predominance
  • Heat intolerance
  • Fine, sparse hair
  • Periocular wrinkling with hyperpigmentation
  • Dystrophic or brittle nails
  • Hypoplastic or absent salivary glands
  • Teeth are markedly reduced in number and have abnormal crown shapes
  • Treatment includes genetic counseling and various dental prosthetics

Question 33

Answer 33

Osteogenesis Imperfecta

• Group of heritable disorders
• Defect in type I collagen maturation
• Most common type of inherited bone disease
• Bone has:
  
  • Thin cortex
  • Fine trabeculation
  • Diffuse osteoporosis
• Upon fracture, healing occurs but may heal inappropriately
• Affects 1:8000
• Signs/symptoms:
  
  • Bone fragility
  • Blue sclera
  • Altered teeth -which look clinically identical to dentinogenesis imperfecta
  • Hearing loss (hypoacusis)
  • Long bone & spine deformities
  • Joint hyperextensiblity
• XRAY features:
• Osteopenia (low bone density)
• Bowing
  
  • Angulation or deformity of long bones – due to lack of cortical and even some trabecular bone.
  • Multiple fractures
  • Wormian bones in the skull (small bones that are often found within the sutures and fontanelles of the skull)
• Distinctive, occasional, findings noted in the oral cavity:
  
  • Teeth with a blue to brown translucence
  • Premature pulpal obliteration
  • Class III malocclusion - usually
• Four major types:
  
  • Type I: Most common, mildest, blue sclera throughout life
  • Type II: Not compatible with life
  • Type III: Most severe form; 1/3 survive into adulthood
  • Type IV: Resembles type I; slightly more severe (more fractures)
• Goal of therapy is symptomatic treatment & management of fractures
• Patients may be on bisphosphonates
• Reserved for moderately to severely affected patients

Question 34

Answer 34

Giant Cell Fibroma

• Fibrous tumor with distinctive features; not associated with chronic irritation
• Typically have a papillary surface
• Occurs at a younger age than other fibromas
• Predilection for the gingiva
• Retrocuspid papilla is a microscopically similar lesion of the gingiva, lingual to mandibular canine
  
  • Typically bilateral, present in up to 99% of children
  • Normal anatomic variation that regresses with age
• Treatment is conservative surgical excision
  
  • Retrocuspid papilla should be recognized clinically; no biopsy is needed – If it is bilateral, you don’t do anything about it, if unilateral, you’re decision to biopsy or not.

Question 35

Answer 35

Odontoma- compound

Question 36

What is the most complicated involvement of Pemphigus vulgaris?

Answer 36

Skin lesions

Question 37

What is another name for erythema multiforme major?

Answer 37

Stevens-Johnson syndrome

Question 38

What part of the body is Burkitt’s Lymphoma found in the majority of the time?

Answer 38

Predilection for the jaws (up to 70% present in the jaws)

Question 39

What are the two most common things that cross the midline? Unlilocular that cross the midline? Multilocular that cross the midline?

Answer 39

1. CGCG, Glandular Odontogenic Cyst
2. Ameloblastoma, OKC, CGCG
3. CGCG (unless associated with a tooth, then this goes to bottom of list), Ameloblastoma, OKC, Glandular Odontogenic Cyst (not associated with a tooth)

Question 40

What are lisch nodules?

Answer 40

They are Iris hamartomas, and two or more of them is one of the diagnostic criteria for NF1.

Question 41

What is the most common cause of death of Lupus?

Answer 41

Renal disease

Question 42

Between what ages are osteosarcomas most common? And in what two locations are they most common in this age group?

Answer 42

Between 10 and 20 years old.

1. Distal Femur
2. Proximal Tibia

Question 43

Answer 43

Multiple Myeloma

• Malignancy of plasma cell origin
• Has a multicentric origin within bone
• If metastatic disease is excluded, then multiple myeloma accounts for 50% of all malignancies that involve bone – Know this
• Abnormal cells are monoclonal; likely arise from a single precursor that undergoes uncontrolled mitotic division
• Signs and symptoms of the disease result from uncontrolled proliferation of the tumor cells and the uncontrolled manufacture of their protein products
• Average age is 65; M>W; AA>C
  
  • Most common hematologic malignancy of AA – Know this
• Bone pain is the most characteristic presenting symptom
  
  • Most common in the lumbar spine
• Some patients may have pathologic fractures, complain of fatigue, or have petechiae
• XRAY features:
  
  • Multiple, well-defined, “punched-out” radiolucencies
  • May be ragged radiolucent lesions
  • Especially evident on skull film
• Renal failure may be a presenting sign because the kidneys become overburdened with the excess circulating proteins
  
  • Up to 50% of patients will multiple myeloma will have these proteins in the urine
    
    • Termed Bence Jones proteins
• Goal of treatment is to make the patient comfortable and control the malignancy
• Chemotherapy is typically used; 60% respond
• Virtually all relapse
• Bisphosphonates are given to reduce fractures
• Prognosis is poor and has a median survival of 3 years

Question 44

Answer 44

Ameloblastoma, OKC, central giant cell granuloma ( multi. Radiolucencys=OKC) gorlin syndrome!!

Question 45

What are the characteristics of Diabetes Mellitus?

Answer 45

• The main focus will be on oral manifestations
• Many complications are due microangiopathy, which results in occlusion of the small blood vessels; a decrease in tissue perfusion results – which can lead to necrosis and amputation
• Patients also have an impairment of neutrophil function
• Oral manifestations are essentially limited to those with type I DM
  
  • Periodontal disease:
    
    • Occurs more frequently and progress more rapidly than in normal patients
  • Delayed healing
  • Increased probability of infection
  • Enlargement and erythema of the attached gingiva
  • Oral candidiasis (30%)
  • Zygomycosis – poorly controlled
  • Xerostomia (30%)
  • Diabetic sialadenosis
  • Diffuse, nontender, bilateral enlargement of the parotid glands
• Treatment is geared toward controlling DM

Question 46

How does a Juvenile (Active) Ossifying Fibroma differ from a Ossifying Fibroma?

Answer 46

On basis of age of patient, most common sites of involvement, and clinical behavior. Juvenile is more common in males, and in maxillary region, while Ossifying Fibroma is more common in Females, and in the mandible.

Question 47

Answer 47

Gingival Cyst of the Adult

• **Represents the soft tissue counterpart of the lateral periodontal cyst** - know this
• Derived from rests of dental lamina (rests of Serres)
• Striking predilection to occur in the mandibular canine & premolar area
• Most commonly found in pts age 40-50
• Invariably located on the facial gingiva or alveolar mucosa
• Painless, dome-like swellings which are less than 5mm
• May be blue in color
• Surgical excision with is sufficient treatment

Question 48

Answer 48

Traumatic bone cyst (simple bone cyst)

Question 49

Answer 49

Cleidocranial Dysplasia

• Syndrome complex characterized by dental and clavicle abnormalities
• 1 in 1,000,000
• usually, the clavicles are present and show varying degrees of hypoplasia
  
  • Clavicles are absent in 10% of cases
  • Unusual mobility of shoulders
• Appearance is diagnostic:
  
  • Short stature; big head
  • Pronounced frontal bossing
  • Ocular hypertelorism
  • Broad base of nose
• Patients have a high-arched palate
• Increased prevanence of cleft palate
• Prolonged retention of deciduous teeth
• Delay or failure of eruption of permanent teeth
• Abnormally shaped teeth
• **Numerous unerupted permanent and supernumerary teeth**
  
  • Up to 60 supernumerary teeth have been reported
• No treatment exists for the bone anomalies, but the patients typically function well
• Patients are usually unaware they have a syndrome
• Treatment of dental problems is difficult
• Can include full-mouth extractions with dentures or various other pros, ortho, & oral surgery related options

Question 50

What percent of patients of Florid Cemento-Osseous Dysplasia are females? And what percentage are African Americans? And what is the average age group?

Answer 50

90%, 90%, and Middle-Aged adults.

Question 51

Answer 51

Crohn’s Disease

• Inflammatory and immunologically mediated condition of unknown cause
• Manifestations can be seen anywhere along the GI tract (from mouth to anus)
• Oral lesions are significant because they precede the GI lesions 30% of the time
• Most patients are teenagers when the disease first becomes evident
• GI signs include abdominal cramping, diarrhea, pain, nausea, and fever
• Weight loss and malnutrition may develop
• Oral lesions are nonspecific:
• Diffuse, nodular swellings
• “Cobblestone appearance” of the oral mucosa
• Linear ulcerations of the buccal vestibule
• Treatment is usually with a sulfa drug
• Systemic prednisone is used in more severe cases
• Oral lesions clear with treatment of GI disease

Question 52

Answer 52

Fibroma

• The most common “tumor” of the oral cavity – Not a neoplasm, doesn’t have an unlimited growth potential.
• Reactive hyperplasia (not neoplasia) of fibrous connective tissue in response to local irritation or trauma
• Most common location is the buccal mucosa along the bite line
• Typically appears as an asymptomatic, sessile, smooth-surfaced nodule that is similar in color to the surrounding mucosa
• Conservative surgical excision is curative
• MUST submit the excised tissue for microscopic examination

Question 53

Answer 53

Langerhans Cell Histiocytosis

• Sharply demarcated (“punched-out”) radiolucencies
• Occasionally may be ill-defined
• Mandibular lesions appear “scooped out” because the superficial alveolar bone is destroyed
• Extensive alveolar bone involvement resembles severe periodontitis clinically and radiographically the teeth look as though they are “floating in air”

Question 54

Answer 54

Hemiangioma

• The most common tumor of infancy
• More common in females (3:1, 5:1), and in whites (versus other racial groups)
• Most common location is the head and neck, which accounts for 60% of all cases
• Types: capillary & cavernous
  
  • Capillary: may not blanch clinically, usually red in color
  • Cavernous: typically blanches, darker red to purple

Question 55

What is primary amyloidosis associated with?

Answer 55

Myeloma

Question 56

What is the biggest worry in Paget’s Disease of Bone?

Answer 56

The development of a malignant bone tumor, osteosarcoma.

Question 57

What is the name of a lateral periodontal cyst that is multilocular?

Answer 57

Botryoid (grapelike) Odontogenic Cyst

Question 58

Notes from Review Sessions

Answer 58

Oral Path Differentials 9/30/15

Test questions

• Rhabdoymyosarcoma
• Leukemia
• Sturge Webers
• Gigantism
• Plummer-Vinson
• ABC/TBC-aneurysmal/traumatic
  
  • Not true cysts
  • TBC: scalloping btw roots, hollow cavity
  • ABC: ballooning expansion of inferior border cortex, blood filled sponge

Mixed RO/RL odontogenic tumor (around teeth)

• CEOT (Older population)
• AOT (Younger population, mx>md)
• COC
• AFO (Younger population, AFàAFOàodontoma)

*Age makes a difference in your differential

Fibroma: The most common benign tumor of oral cavity

• Once it’s on the gumsà3Ps/I differential

Unilocular RL, crossing anterior midline, md

• Ameloblastoma
• OKC
• CGC

Multilocular RL, crossing anterior midline, md

• CGC (not associated w/ a tooth. If it is then it drops to the bottom of the list and Ameloblastoma and OKC move to the top)
• Ameloblastoma
• OKC
• Glandular Odontogenic Cyst (not associated w/ a tooth)

*Differential: see if a tooth is involved or not

Unilocular RL surrounding the crown of an impacted tooth

• Dentigerous cyst (or Eruption cyst)
• OKC
• Ameloblastoma (Can be unicystic or multicystic)
• CGC

*tooth involved

The only clinical picture he would show from Ch 17 for PCP would be atrophic glossitis. Be able to give one possible cause of it.

Things to know about SLE: malar rash, arthritis, kidney problems, tx w/ antimalarials (hydroxychloroquine turns the palate blue)

A child w/ bilateral multilocular RL in the rami of the mandible.

• Clinical dx: CGC
• Syndromic dx: Cherubism

Question 59

Answer 59

Central Giant Cell Granuloma

• The histology is a cellular, vascular stroma with prominent multinucleated giant cells (Without the history or the radiograph, this image looks like other things, like a peripheral giant cell granuloma)

Question 60

With what unerupted tooth is the adenomatoid odontigenic tumor most common?

Answer 60

Canines usually, help distinguish from dentigerous cysts and stuff. And RL often extends apically past the CEJ, which is different. Also has snowflake calcifications via XRAY.

Question 61

Answer 61

Peutz-Jeghers Syndrome

• Rare but well-recognized condition
• A system complex characterized by:
  
  • Freckle-like lesions of the hands, perioral skin, and oral mucosa
  • Intestinal polyposis – the polyps of this disease are not malignant, but the polyps of Gardner’s will develop into cancer, so the treatment with Gardner’s is prophylactic colectomy.
  • Predisposition for affected patients to develop cancer
• Generally AD inherited
• Skin lesions develop in childhood and involve periorificial areas
  
  • Lesions resemble freckles, but do not wax and wane with sun exposure
• Intestinal polyps are hamartomatous growths
  
  • Not premalignant
  • Scattered throughout the mucous-producing areas of the GI tract
• Patients also have a problem with intestinal obstruction due to intussusception
  
  • “Telescoping” of a proximal segment of the bowel into the distal portion
• Patients are about 18x more likely to have a malignancy in their lifetime
  
  • GI adenocarcinoma also develops (but not from the polys)
  • Other tumors occur such as breast cancer
• Oral lesions are an extension of the perioral freckling
  
  • They are 1-4 mm brown macules
  • Primarily affect the labial mucosa, buccal mucosa, and tongue
  • Seen in 90%
  • Treatment for these patients is monitoring for development of intussusception or tumors

Question 62

Answer 62

Lymphangioma

• Benign tumor of lymphatic vessels
• Types: capillary, cavernous, cystic
• Cavernous lymphangiomas are more frequently found in the mouth
• Cystic lymphangiomas (cytic hygroma) most often occur in the neck
• Marked predilection for head and neck, accounts for 50%-75% of all cases
• Half of all lesions are noted at birth, 90% develop by 2 years of age
• Oral lymphangiomas occur most frequently on the anterior two thirds of the tongue, where they often result in macroglossia
• Clinically can look like “frog eggs” or “tapioca pudding”

Question 63

What pathology is characteristic of a downward bowing of the inferior cortex of the mandible?

Answer 63

Ossifying Fibroma. Ameloblastomas and Traumatic Bone Cysts also have cortical expansion, which is similar.

Question 64

What is the distance that normally separates the REE and tooth crown?

Answer 64

3mm. If fluid gets in and it gets bigger, the dentigerous cyst will grow, and the lining is stratified squamous epithelium.

Question 65

Where is the most common location in the mouth for the lateral periodontal cyst?

Answer 65

Mandibular premolar-canine-lateral incisor area.

Question 66

What pathology is a true mixed tumor, meaning both epithelial and mesenchymal tissues are neoplastic?

Answer 66

Ameloblastic Fibroma

Question 67

Answer 67

Melanotic Neuroectodermal Tumor of Infancy

• Rare pigmented tumor (babies are born with it)
• Usually occurs during the 1st year of life
• Striking predilection for anterior maxilla (61%) – know this
• Rapidly expanding mass
• Clinically black and/or blue
• High urinary levels of vanillylmandelic acid (VMA)
• Most are benign

Question 68

What is the prognosis for SLE and CCLE?

Answer 68

• SLE:
  
  • 5-year survival is 90%; 20-year survival is 70%
  • Most common cause of death is renal failure
  • Prognosis is better for women and Caucasians when compared to men and African Americans
• CCLE:
  
  • Much better prognosis than SLE, but can be a nuisance
  • 50% of cases resolve spontaneously
  • 5% progress to SLE

Question 69

Answer 69

Lateral periodontal cyst ( botryoid-grapelike)

Question 70

What do CEOT’s secrete, and what is it positive for? And what are the names of the calcifications with concentric rings that form in the amyloid-like areas?

Answer 70

• They secrete amyloid-like extracellular material; positive for Congo red, which exhibits an apple-green birefringence when viewed under polarized light
• Liesegang rings

Question 71

Answer 71

Gardner Syndrome

• A symptom complex characterized by: - their polyps will result in cancer
• Colonic polyps/adenocarcinoma
• Skeletal abnormalities (90%)
• Dental abnormalities (20%)
• Epidermoid cysts
• Dermoid tumors (10%)
• Thyroid carcinoma
• Pigmented lesions of the ocular fundus (90%)

Present in 1:10,000

• Bowel polyps:
  
  • Develop during teenage years
  • **WILL transform into adenocarcinoma**
    
    • 50% by age 30
    • 100% in ‘older’ patients
• Skeletal abnormalities:
  
  • **Osteomas are most common**
    
    • Noted during puberty
  • Affect the skull, paranasal sinuses, and mandible
  • Precede the development of bowel polyps
  • Most patients have between 3-6 lesions
• Dental abnormalities:
  
  • **Supernumerary teeth** - the only other one it could be is cleidocranial dysplasia, and both answers would be right.
  • Impacted teeth
  • Odontomas
  • Treatment is prophylactic colectomy; long-term prognosis depends on the development and behavior of bowel adenocarcinomas

Question 72

Answer 72

Not Sure

Question 73

Answer 73

Odontoma (Complex)

• Most common odontogenic tumor
• Considered the late-stage of ameloblastic fibroma
• Considered to be developmental anomalies (hamartomas) rather than true neoplasms
• Divided into compound and complex types
  
  • Compound – Composed of multiple, small, toothlike structures
  • Complex- Conglomerate mass of enamel and dentin; bears no anatomic resemblance of a tooth
• Ave age – 15
• Completely asymptomatic
• Relatively small and discovered via XRAY when films are taken to determine the reason for failure of tooth eruption
  
  • Usually associated with an unerupted tooth
• Compound is more common in anterior maxilla; complex is more common in the molar regions – he won’t ask us this.
• Compound odontoma, via XRAY:
  
  • Appears as a collection of toothlike structures of varying size and shape
  • Surrounded by a narrow RL zone
• Complex odontoma, via XRAY:
  
  • Calcified mass with the radiodensity of a tooth
  • Surrounded by a narrow, RL rim
• XRAY findings are usually diagnostic
• Odontomas are treated by simple local excision with an excellent prognosis

Question 74

Answer 74

Calcifying Epithelial Odontogenic Tumor

• **AKA Pindborg Tumor**
• Occurs around age 40
• M=F; md>mx; posterior>anterior
• Most common presenting sign is a painless, slow-growing swelling
• Via XRAY:
  
  • Unilocular or multilocular
    
    • Unilocular is more common in the maxilla
  • Margins are typically scalloped & well-defined; may be corticated or ill-defined
  • Frequently associated with an impacted tooth (most often a mandibular molar)
  • Contains calcified structures of varying size and density
    
    • Some believe the calcifications are prominent around the crown in a “driven-snow” pattern
    • Occurs in 10%
• Histopathologic features: - know these histologic features, we won’t need to identify them though.
• Nuclear pleomorphism & atypia
• They secrete amyloid-like extracellular material; positive for Congo red, which exhibits an apple-green birefringence when viewed under polarized light
• Calcifications with concentric rings form in the amyloid-like areas (Liesegang rings)
• Conservative local resection with a narrow rim of bone is the treatment of choice
• Recurrence rate is 15%; highest with curettage as treatment
• Prognosis is typically good, but rare lesions can exhibit aggressive or malignant behavior

Question 75

What is the most common hematologic malignancy of African Americans?

Answer 75

Multiple Myeloma. Average age is 65.

Question 76

Which pathology is associated with displacing a tooth way up near the orbit?

Answer 76

Odontogenic Keratocyst

Question 77

What are nuclear pleomorphism and atypia associated with when it comes to histopathologic features?

Answer 77

Calcifying Epithelial Odontogenic Tumor

Question 78

Answer 78

Periapical Cemento-Osseous Dysplasia

• Involves the periapical region of the anterior mandible
• Multiple foci are usually present
• 90% are female; 70% in African Americans
• Average age = 40 – same thing, middle-aged women
• Teeth are invariably vital
• Asymptomatic and discovered when XRAYs are taken for other reasons
  
  • Early lesions are circumscribed areas of RL involving the apex of a tooth – this lesion looks identical to that of a periapical granuloma or cyst
  • Lesions “mature” over time to have a mixed RL-RO appearance
  • End-stage lesions are densely RO with a RL rim
    
    • The PDL will be intact; the lesion will NOT fuse to the tooth
    • Each lesion is self-limiting and progressive growth does not occur

Question 79

What is numb-chin syndrome associated with?

Answer 79

Metastatic tumors to the jaw, involving the inferior alveolar nerve, producing a distinctive pattern of anesthesia.

Question 80

Answer 80

Buccal bifurcation cyst

Question 81

Answer 81

Question 82

What is the most common site of Granular Cell Tumors?

Answer 82

The tongue (50%), usually dorsal side, so benign.

Question 83

Where is the most common site for pyogenic granulomas in the mouth? And in what age groups is this most common?

Answer 83

Gingiva (75%). Most common in children and young adults.

Question 84

How can you differentiate Idiopathic Osteosclerosis from Condensing Osteitis? From Focal Cemento-Osseous Dysplasia? From Cementoblastoma?

Answer 84

Condensing osteitis will be associated with an infection, and the tooth will have deep caries or a deep filling or root canal. Focal Cemento-osseous dysplasia will have a radiolucent rim. Cementoblastoma will be fused with the tooth.

Question 85

Answer 85

Florid cement osseus dysplasia

Question 86

Where is the most common site for a fibrous histiocytoma when in the oral cavity?

Answer 86

Buccal mucosa

Question 87

What is the other lesion of the gingiva that is microscopically similar to a giant cell fibroma, lingual to mandibular canines?

Answer 87

Retrocuspid papilla

Question 88

Answer 88

Peripheral Ossifying Fibroma

• Occurs exclusively on the gingiva – Usually tissue colored
• Appears as a nodular mass, usually originating from the dental papilla
• Average age is 15 (teenagers and young adults 10-19)
• 2/3 occur in females
• 50% of cases occur in the incisor-cuspid region
• Treatment is excision down to periosteum and scale adjacent teeth
• 15% recur (usually because the base of the lesion was not removed)

Question 89

Answer 89

Burkitt’s Lymphoma

• Malignancy of B-cell origin
• First documented in African children
  
  • Termed African Burkitt’s lymphoma
    
    • Because it can be seen in other areas of the world, some prefer to call it endemic Burkitt’s lymphoma
• Predilection for the jaws (up to 70% present in the jaws)
• Over 90% are positive for EBV
• American Burkitt’s lymphoma (or sporadic) have been observed and usually appear as an abdominal mass
• Average age is 7; M>F – more common in children
• Posterior jaws>anterior; maxilla>mandible
• Growth of the tumor mass may produce facial swelling and proptosis
• XRAY features:
  
  • Consistent with a malignant process
    
    • RL destruction of the bone with ragged, ill-defined margins - not corticated
    • May begin as several smaller sites, which eventually enlarge and coalesce
    • Patchy loss of the lamina dura is an early sign
• Histopathology:
  
  • Low-power magnification will show the classic “starry-sky” pattern, which is due to macrophages within tumor tissue
• Aggressive malignancy; the untreated patient will succumb to the disease in 6 months
• Treatment is intensive chemotherapy
• Prognosis is 90% 5 year survival rate for Stage I or II; 80% for stage III or IV
• Due to aggressive treatment

Question 90

Answer 90

Osteosarcoma

Question 91

Answer 91

SLE

• Difficult to diagnose in early stages because it is nonspecific and has periods of remission
• F:M 10:1; avg age = 30
• Common findings:
  
  • Fever
  • Weight loss
  • Arthritis
  • Fatigue
  • Malar rash – 50%
    
    • Characteristic rash with the pattern of a butterfly that develops of the malar area and nose but spares the nasolabial folds
    • Sunlight makes the lesion worsen
• Kidneys are affected in up to 50%
  
  • This complication may lead to kidney failure and is the most significant aspect of the disease – Renal disease is the most common cause of death of Lupus
• Cardiac complications:
  
  • Most common = pericarditis
  • Up to 50% have warty vegetations affecting heart valves termed Libman-Sacks endocarditis
• Oral lesions are seen in up to 40%
  
  • Nonspecific
  • May appear as lichenoid lesions

Question 92

Vitamin E name and function?

Answer 92

Vitamin E (α-tocopherol): antioxidant.

Question 93

Which disease is associated with a cotton wool or cotton roll appearance on XRAYs?

Answer 93

Paget’s Disease of Bone

Question 94

What are the two types of odontomas?

Answer 94

1. Compound – Composed of multiple, small, toothlike structures
2. Complex- Conglomerate mass of enamel and dentin; bears no anatomic resemblance of a tooth

Question 95

What is Codman’s Triangle?

Answer 95

It is a triangular elevation of periosteum that can form with osteosarcoma.

Question 96

Where does a peripheral ossifying fibroma exclusively occur?

Answer 96

On the gingiva, and it is usually always tissue colored.

Question 97

List of Odontogenic Tumors

Answer 97

Tumors of Odontogenic Epithelium

• Odontogenic epithelial origin:
• Ameloblastic Carcinoma
• Malignant Ameloblastoma
• Adenomatoid Odontogenic Tumor (AOT) – He will expect us to write this out.
• Calcifying Epithelial Odontogenic Tumor (CEOT)
  
  • AKA Pindborg tumor

Mixed odontogenic tumors:

• Ameloblastic Fibroma
• Ameloblastic Fibro-odontoma
• Ameloblastic Fibrosarcoma
• Odontoma
• Odontogenic tumors

Odontogenic ectomesenchyme tumors:

• Central Odontogenic Fibroma (COF)
• Peripheral Odontogenic Fibroma
• Odontogenic Myxoma
• Cementoblastoma

Question 98

Answer 98

Plasmacytoma

• Unifocal, monoclonal neoplastic proliferation of plasma cells
• Typically arises in bone
• Probably gives rise to multiple myeloma
• Shares all the same demographics, signs, and symptoms as MM
• 50% will have multiple myeloma within 2-3 years

Question 99

What is the most common cardiac complication in lupus?

Answer 99

Pericarditis (libman-sacks endocarditis)

Question 100

Answer 100

Erythema Multiforme

• Blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis
  
  • Likely immunologically mediated
• In 50% of cases, the clinician can identify a preceding infection or exposure to a medication
  
  • In minor, Infection is usually herpes simplex or Mycoplasma pneumoniae; medications are usually antibiotics or analgesics
• A spectrum of the disease exists:
  
  • EM minor, EM major (AKA Stevens-Johnson syndrome) and toxic epidermal necrolysis (AKA Lyell’s disease)
• Acute onset with a wide spectrum of clinical disease
  
  • In mild cases, ulcerations affecting the oral mucosa develop
  • In severe cases, the entire skin & mucosal surfaces may slough
• Patients are usually young adults in their 20s & 30s; M>F
• Prodromal symptoms (fever, headache, cough, sore throat) occur 1 week before onset
• Disease lasts 2-6 weeks; 20% have recurrent episodes
• 50% of pts will develop skin lesions
• Typically, early lesions are flat, round, and red
  
  • Lesions become elevated and evolve into bulla with necrotic centers
  • A highly characteristic skin lesion that develops is a target lesion; these are concentric circular erythematous rings resembling a target or bull’s-eye
• Oral lesions are erythematous patches that undergo epithelial necrosis and ulcerate
• Entire oral cavity can be involved except the gingiva and hard palate
• Hemorrhagic crusting of the vermilion zone of the lips is common
• The lesions emerge quickly and are painful
• Patients may become dehydrated due to an inability to ingest liquids
• Steven-Johnson syndrome is known as erythema multiforme major – he wouldn’t show us a picture of this, but will of a minor form, which we will need to know.
  
  • Usually triggered by a drug
  • To make this diagnosis, there must be skin, oral mucosa, and either ocular or genital mucosa involvement
• Toxic Epidermal Necrolysis:
  
  • Most severe form
  • Almost always triggered by a drug
  • Diffuse sloughing of the skin and mucosa
  • Tends to occur in older people (EM minor and major are younger people)
  • F>M (unlike EM minor)
  • If the patient survives, the cutaneous process resolves in 2-4 weeks
• Management of EM is controversial
  
  • Discontinuation of the causative drug is paramount
  • If due to herpes, daily antivirals are indicated
  • Patient is usually admitted to hospital for IV fluids
• Steroids are used in EM minor & major but are contraindicated in TEN
• TEN patients are treated in the burn unit
• Mortality for EM major is up to 10%
• Mortality for TEN is about 35%

Question 101

What does a peripheral ameloblastoma look like?

Answer 101

A bump on the gums, looks like one of the three Ps.

Question 102

What is the most common region of the mouth for Cementoblastomas?

Answer 102

75% arise in the mandible, almost always in the molar/premolar region. And typically only affect permanent teeth.

Question 103

For which types of cemento-osseous dysplasia can you diagnose on an XRAY and not need a biopsy? And which one definitely contraindicates a biopsy?

Answer 103

• For periapical- or florid cemento-osseous dysplasia, diagnosis can be made from the distinctive clinical & XRAY findings – do NOT need biopsy
• In fact, biopsy of florid COD may lead to necrosis due to the hypovascularity
• Focal may require surgical investigation because it is less specific.

Question 104

Answer 104

Neurofibromatosis Type I (NF1)

• Neurofibromatosis is a hereditary condition affecting 1 in 3000
• NF1 is the most common of the 8 recognized forms
• NF1 is also termed von Recklinghausen’s disease of the skin
• AD
• Plexiform variant of NF is pathognomonic
• Pathognomonic = if you have it, you have the disease
• Plexiform NF feels like a “bag of worms”
• 2/3 of patients have mild disease
• Diagnostic criteria (2 or more):
  
  • Six or more café au lait (coffee and milk) macules know this
    
    • “Coast of California” know this
  • Two or more NF or one plexiform NF
  • Freckling in the axillary region (Crowe’s sign) know this
  • Optic glioma
  • Two or more iris hamartomas (Lisch nodules) know this
  • Osseous lesion such as sphenoid dysplasia
  • First-degree relative with NF1
• Oral manifestations occur in 90%:
  
  • Enlargement of the fungiform papilla in 50%
  • Intraoral neurofibromas in 25%
  • Enlargement of the mandibular foramen or canal
• Treatment is directed toward prevention or management of complications
• Up to 5% have malignant peripheral nerve sheath tumors (MPNST) with an associated 5-year survival of 15% - This is the complication he would ask
• Other various malignancies are associated with NF1
• Genetic counseling is extremely important

Question 105

Answer 105

Odontoma (Compound)

• Most common odontogenic tumor
• Considered the late-stage of ameloblastic fibroma
• Considered to be developmental anomalies (hamartomas) rather than true neoplasms
• Divided into compound and complex types
  
  • Compound – Composed of multiple, small, toothlike structures
  • Complex- Conglomerate mass of enamel and dentin; bears no anatomic resemblance of a tooth
• Ave age – 15
• Completely asymptomatic
• Relatively small and discovered via XRAY when films are taken to determine the reason for failure of tooth eruption
  
  • Usually associated with an unerupted tooth
• Compound is more common in anterior maxilla; complex is more common in the molar regions – he won’t ask us this.
• Compound odontoma, via XRAY:
  
  • Appears as a collection of toothlike structures of varying size and shape
  • Surrounded by a narrow RL zone
• Complex odontoma, via XRAY:
  
  • Calcified mass with the radiodensity of a tooth
  • Surrounded by a narrow, RL rim
• XRAY findings are usually diagnostic
• Odontomas are treated by simple local excision with an excellent prognosis

Question 106

What are the three histopathologic features of Ameloblastomas?

Answer 106

1. *Palisading**, hyperchromatic basal layer – odontogenic keratocyst also has hyperchromatic
2. **Reverse polarity** - nucleus is on opposite side of where it usually is
3. You also have a red, white, and blue layer sequence on histology

-Know how to differentiate this histology from other ones, especially OKC, with Ameloblastoma, there is a lot more tissue, with OKC, it’s usually thinner areas and 6-8 layers.

Question 107

Answer 107

Ameloblastic Fibro-Odontoma

• Histologically appears similar to ameloblastic fibroma, except it also has enamel and dentin
• We believe that these tumors start as an ameloblastic fibroma, become ameloblastic fibro-odontomas, and then just become odontomas.
• Avg age is 10
• Occurs in the posterior jaws
• Small lesions are asymptomatic; large lesions are associated with a painless swelling
• Via XRAY:
  
  • Well-circumscribed unilocular radiolucency
  • Contains varying amounts of calcified material
  • Typically associated with an unerupted tooth
  • Treated with conservative curettage with rare recurrence

Question 108

Where is the most common location of an oral focal mucinosis?

Answer 108

• 2/3 occur on the gingiva, 1/3 on hard palate
• Results from overproduction of hyaluronic acid by fibroblasts

Question 109

With what cancer is plummer-vinson syndrome highly associated with?

Answer 109

Oral and esophageal squamous cell carcinoma

Question 110

Vitamin D name and function?

Answer 110

Vitamin D: considered a hormone, necessary for calcium absorption from the gut. Rickets, Osteomalacia.

Question 111

What are wormian bones and what disease are they associated with?

Answer 111

They are small bones in the skull that are often found within the sutures and fontanelles, and are associated with Osteogenesis Imperfecta.

Question 112

What is another name for NF1?

Answer 112

Von Recklinghausen’s disease of the skin

Question 113

What are high urinary levels of vanillylmandelic acid associated (VMA) with?

Answer 113

Melanotic Neuroectodermal Tumor of Infancy

Question 114

What are more common with Osteomas, paranasal sinus lesions or gnathic lesions?

Answer 114

Paranasal sinus lesions

Question 115

Where are cavernous lymphangiomas mostly found and where are cystic lymphangiomas mostly found? Most common site for oral cases? What does it look like?

Answer 115

Cavernous - in mouth. Cystic - in neck. Oral lymphangiomas occur most frequently on the anterior two thirds of the tongue, resulting in macroglossia. Clinically can look like “frog eggs.”

Question 116

Answer 116

Lipoma

• Benign tumor of fat
• By far the most common mesenchymal (non-epithelial, so soft tissue) neoplasm – Know this, but more common on the trunk
• More common in obese patients, but the metabolism is independent of normal body fat
• Appear as soft, smooth-surfaced nodular masses
• Lesion may be yellow or mucosal-colored or white
• 50% occur in the buccal mucosa
• Most patients are older than age 40
• Float in formalin (helps with diagnosis)
• Surgical excision is curative

Question 117

Where in the mouth does the desmoplastic ameloblastoma occur most commonly?

Answer 117

Anterior Maxilla, and may be mixed RO and RL, similar to calcifying odontogenic cyst.

Question 118

Answer 118

desmosomes, pemphigus vulgaris(histo picture)

Question 119

What is the most common lymphoma in the mouth? And what percent are B-lymphocyte in origin?

Answer 119

Non-Hodgkins Lymphoma, specifically, Diffuse Large B Cell (DLBCL). 80% are of B-lymphocyte origin.

Question 120

What pathology represents the soft tissue counterpart of the lateral periodontal cyst?

Answer 120

Gingival Cyst of the Adult

Question 121

Answer 121

periapical cement-osseaus dysplasia (middle age female, A.A> caucasains) except focal.

Question 122

What are the four skin lesions (the 4 P’s) of Lichen Planus?

Answer 122

1. Purple
2. Pruritus - itchy
3. Polygonal
4. Papules – slightly raised

Question 123

Answer 123

Osteopetrosis

Question 124

In what area of mouth is congenital epulis most common?

Answer 124

Maxillary alveolar ridge where developing lateral incisor-canines are coming in. 90% occur in females.

Question 125

Answer 125

Osteopetrosis

• AKA “marble bone disease”
• Skeletal disorder with a marked increase in bone density
• Results from a defect in remodeling caused by a failure of normal osteoclast function
• Two major clinical patterns:
  
  • Infantile
  • Adult
• Osteopetrosis - Infantile
  
  • Patients with osteopetrosis discovered at birth or early infancy = malignant osteopetrosis
  • Severe disease
  • Results in:
    
    • Marrow failure
    • Frequent fractures
    • Cranial nerve compression
    • Facial deformities
    • Delayed tooth eruption
    • Osteomyelitis post tooth infraction
• Osteopetrosis - Infantile
  
  • Via XRAY:
    
    • Widespread increase in skeletal density (increased radiopaque)
    • Roots of teeth are difficult to visualize because of the density of the surrounding bone
• Osteopetrosis - Adult
  
  • Discovered later in life and has less severe manifestations
  • AKA benign osteopetrosis
  • 40% are asymptomatic
  • Marrow failure is rare
• Significant complications include fracture & osteomyelitis after tooth extraction
• Prognosis is poor for the infantile form
• Most die in first decade of life
• Bone marrow transplant is only hope; only 50% of those eligible survive the transplant
• Adult osteopetrosis is mild and has long-term survival

Question 126

What are the characteristics of Vitamin Deficiencies?

Answer 126

• In the U.S., significant vitamin deficiencies are not common
• Patients with malabsorption syndromes or eating disorders, those following “fad diets,” and alcoholics are most commonly affected.
• Vitamin A (retinol): essential for vision. May lead to blindness.
• Vitamin B1 (thiamin): maintain proper functioning of neurons. Beriberi.
• Vitamin B2 (riboflavin): necessary for cellular oxidation-reduction reactions. Oral alterations like angular cheilitis, glossitis.
• Vitamin B3 (niacin): acts as a coenzyme for oxidation-reduction reactions. Pellagra= dermatitis, dementia, diarrhea.
• Vitamin B6 (pyridoxine): cofactor associated with enzymes that participate in amino acid synthesis.
• Vitamin C (ascorbic acid): necessary for the proper synthesis of collagen. Scurvy**.
• Vitamin D: considered a hormone, necessary for calcium absorption from the gut. Rickets, Osteomalacia.
• Vitamin E (α-tocopherol): antioxidant.
• Vitamin K: necessary for proper clotting (needed in clotting factors II,VII, IX, X). Coagulopathy because of inadequate synthesis or prothrombin.

Question 127

What is the most common tumor of the oral cavity?

Answer 127

Fibroma

Question 128

Answer 128

Pemphigus

• The term ‘pemphigus’ represents four related diseases
  
  • Pemphigus vulgaris
  • Pemphigus vegetans
  • Pemphigus erythematosus
  • Pemphigus foliaceus
• Our discussion is limited to pemphigus vulgaris, the most common of the disorders and affects the oral mucosa
• Oral lesions are the first sign of the disease and the last to resolve with therapy
  
  • “First to show, last to go”
• Autoantibodies are directed against the desmosomes
  
  • Desmosomes bond epithelial cells to each other
  • Therefore, there is a split within the epithelium (intraepithelial split)
• Initial manifestations will affect the oral mucosa in adults, around age 50
• M=F
• Patients complain of oral pain and will exhibit multiple erosions throughout on intraoral examination
• Patients rarely report vesicle or bulla formation intraorally
  
  • Likely due to early rupture because of the thin roof of the blisters
• Skin lesions are flaccid vesicles and bullae that rupture quickly and leave an ulceration
• Skin lesions are the most complicated involvement
• Without treatment, the oral & cutaneous lesions are progressive
• A characteristic feature is a positive Nikolsky sign: Bulla can be induced on normal-appearing skin if firm lateral pressure is exerted
• Histopathologic features:
  
  • Cells of the epithelium “fall apart” – termed acantholysis
  • The loose cells are rounded and termed Tzanck cells, which can be useful in diagnosis
    
    • Remember, the Tzanck cells aren’t exclusive to pemphigus vulgaris – this is also a histopathologic feature of herpes
• Direct immunofluorescence (DIF) is positive for C3 and IgG within the epithelium
• Indirect immunofluorescence (IIF) correlates with disease activity
• Before the development of corticosteroids, 90% died due to infection and electrolyte imbalances
  
  • Today, treatment is to send to a dermatologist, who will administer systemic corticosteroids
  • This is often in combo with other immunosuppressive drugs
    
    • 75% will have disease resolution in 10 years
    • 15% will remain on steroids throughout life
    • 10% succumb to disease, often due to complications of long-term steroid use
    • Hailey-Hailey Disease has “test-tube” like ridges, and it is also called Familial Pemphagus

Question 129

In what age group does Adenomatoid Odontogenic Tumors occur most often?

Answer 129

Young, ages 10-20. **Asymptomatic and discovered during XRAY to determine why a tooth hasn’t erupted**

Question 130

What are the histopathologic “saw-toothed” rete ridge associated with?

Answer 130

Lichen Planus

Question 131

What is Wickhan’s Striae and what is it associated with?

Answer 131

Lichen Planus (reticular), where the posterior buccal mucosa is involved bilaterally.

Question 132

Answer 132

Eruption cyst

Question 133

Which chromosome and gene is associated with Gorlin Syndrome?

Answer 133

Chromosome 9, PTCH gene

Question 134

Excluding hematopoietic neoplasms (leukemia, lymphoma), what is the most common type of malignancy to originate within bone?

Answer 134

Osteosarcoma

Question 135

What are the first signs of the disease and last to resolve with therapy when it comes to Pemphigus vulgaris?

Answer 135

Oral lesions. First to show, last to go.

Question 136

Answer 136

Hodgkin’s Lymphoma

• Malignant lymphoproliferative disorder
• Unlike most malignancies, the neoplastic cells make up only 1-3% of the cells in the lesion
  
  • Neoplastic cells are Reed-Sternberg cells
    
    • Typically binucleated (“owl-eye” nuclei)
• A significant percentage of Hodgkin’s lymphoma is linked to the Epstein-Barr virus (EBV)
• Process almost exclusively begins in the lymph nodes; any node is susceptible
• 75% occur in the cervical and supraclavicular nodes
• Males are more commonly affected than females
• There is a bimodal pattern of age at diagnosis:
  
  • Between 15-35
  • After age 50
• Usual presenting sign is a persistently enlarging, nontender, discrete mass or masses in a lymph node region
• Early lesions are movable, late lesions are more matted and fixed
• Two types of Hodgkin’s Lymphoma:
  
  • Nodular lymphocyte-predominant
  • Classical
• The Classical has five histologic subtypes:
  
  • Lymphocyte rich
  • Nodular sclerosis – F>M; 70% of cases
  • Mixed cellularity
  • Lymphocyte depletion
  • Unclassifiable
  • • Histological Types
      
      • Lymphocyte predominant (7%) - best prognosis
      • Nodular sclerosing (64%) - worst prognosis
      • Mixed cellularity (25%)
      • Lymphocyte depleted (4%)
  • After 15 years posttreatment, patient mortality is due typically to the complications of therapy (secondary malignancy or cardiovascular disease)

Question 137

What is PEN?

Answer 137

Palisaded Encapsulated Neuroma. it is a benign neural tumor typically found on the face (90%)

Question 138

Answer 138

OKC (tooth displaced)

Question 139

What is typically the first sign of MEN, type 2B?

Answer 139

• Oral mucosal neuromas.
• Present as soft, painless papules (more superficial located) or nodules (deeper in connective tissue, but both are still bumps)
• Bilateral neuromas of the commissural mucosa are highly characteristic – Know this

Question 140

Answer 140

Orthokeratinized Odontogenic Cyst

• An odontogenic cysts with orthokeratinized lining and NO basal palisading
  
  • Remember, OKC has parakeratin and a palisaded basal layer – Also, the granular cell layer is present in orthokeratinized, but not in parakeratinized, so it is found here, but not in odontogenic keratocyst.
• Occur in young adults
• 2:1 M:F
• Most common in the posterior mandible – most of these occur in the posterior mandible, so I would remember the ones that DON’T occur in the posterior mandible.
  
  • Typically unilocular
  • Usually appear radiographically as a dentigerous cyst around a third molar
  • Rarely recur after enucleation with curettage (around 2%)

Question 141

List of Odontogenic Cysts

Answer 141

• Dentigerous Cyst
• Eruption Cyst
• Odontogenic Keratocyst (OKC)
  
  • WHO: Keratocystic Odontogenic Tumor (KOT)
  • Associated: Gorlin syndrome
• Orthokeratinzed Odontogenic Cyst (OOC)
• Gingival Cyst of the Adult
• Lateral Periodontal Cyst (LPC)
• Calcifying Odontogeinc Cyst (COC)
  
  • AKA Gorlin Cyst
  • WHO: Calcifying Cystic Odontogenic Tumor
• Glandular Odontogenic Cyst (GOC)
• Buccal Bifurcation Cyst
• Carcinoma arising in Odontogenic Cysts

Question 142

Answer 142

Leukemia

• The leukemic cells may infiltrate the oral soft tissues to produce a boggy, non-tender swelling – Can look like drug-induced gingival hyperplasia, but when you palpate it, it is fairly soft. The classica gingival leukemia looks dark and black and kind of like melanoma, but it is so diffuse so it is easier to differentiate between melanoma and Kaposi’s sarcoma. And with this picture, you would think salivary gland neoplasm until you palpate, and if it is soft, most likely not neoplasm, and this picture is one of Leukemia.

Question 143

Answer 143

Question 144

What do odontogenic keratocysts arise from?

Answer 144

Cell rests of the dental lamina

Question 145

Vitamin K name and function?

Answer 145

Vitamin K: necessary for proper clotting (needed in clotting factors II,VII, IX, X). Coagulopathy because of inadequate synthesis or prothrombin.

Question 146

Answer 146

Gardner Syndrome

**Supernumerary teeth** - the only other one it could be is cleidocranial dysplasia, and both answers would be right.

Question 147

What are the four main characteristics of Gorlin Syndrome?

Answer 147

1. Multiple Basal Cell Carcinomas
2. Odontogenic Keratocysts (OKC)
3. Calcification of the falx cerebri
4. Rib anomalies (bifid ribs)

Question 148

What are the characteristics of Carcinoma Arising in Odontogenic Cysts?

Answer 148

• Carcinoma arising within bone is rare and essentially limited to the jaws – wherever we have epithelium we can get cancer.
• Collectively known as odontogenic carcinomas
  
  • Most arising from odontogenic cysts
• Via XRAY, they mimic odontogenic cysts, except the margins are irregular and ragged
• Histology usually shows well-differentiated SCCA
• Treatment varies from local block excision to radical resection, +/- radiation and chemo
• Approximately 50% survive after treatment
• Must rule out metastatic spread from another area before calling it a true primary intraosseous carcinoma

Question 149

Answer 149

Schwannoma (Neurilemoma)

• Benign neural neoplasm of Schwann cell origin
• Up to half of cases occur in H&N
• Slow-growing, encapsulated tumor which arises in association with a nerve trunk
• Typically asymptomatic
• Most common in young and middle-aged adults
• Bilateral schwannomas of auditory-vestibular nerve are a characteristic feature of the neurofibromatosis type II (NF2) - know for test
  
  • Autosomal dominant
  • Mutation of a tumor suppressor gene on chromosome 22 (merlin)
  • Features: “acoustic neuromas” of vestibular nerve, meningiomas and ependymomas of the central nervous systems
  • Symptoms: deafness, dizziness, and tinnitus
• Tongue is the most common location for oral lesions
• Histopathologic features:
  
  • Antoni A – Streaming fascicles of spindle-shaped Schwann cells which form a palisaded arrangement around central acellular, eosinophilic areas known as Verocay bodies - Organized
  • Antoni B – Basically everything that’s not Antoni A - Disorganized

Question 150

What is the Philadelphia chromosome as it relates to Leukemia, and which type of Leukemia?

Answer 150

• Chronic myeloid leukemia (CML) has a translocation of the chromosomal material between the long arms of chromosomes 9 & 22
• This is termed the Philadelphia chromosome

Question 151

Answer 151

Systemic Sclerosis

• A disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts
• F:M 5:1; disease of adults
• First sign of disease is Raynaud’s phenomenon:
  
  • Vasoconstrictive event triggered by emotional distress or exposure to cold – the tips of the fingers go white and you get that numb sensation
  • Not specific for systemic sclerosis
• Patients also experience resorption of the terminal phalanges and flexion contractures to produce shortened, clawlike fingers
• Vascular events and abnormal collagen deposition contribute to fingertip ulcerations
• Skin develops a diffuse, hard texture with a smooth surface
  
  • Involvement of the facial skin results in a characteristic facial appearance
    
    • Subcutaneous collagen deposition results in smooth, taut, masklike facies
• Nasal alae become atrophied, resulting in a pinched appearance
• Involvement of organs are serious; can lead to fibrosis of:
  
  • Lung
  • Heart
  • Kidney
  • GI tract
• Involvement of organs leads to their failure within 3 years
• Pulmonary fibrosis leads to pulmonary HTN and heart failure
  
  • This is the primary cause of death for these patients
• Microstomia occurs as a result of collagen deposition in the perioral tissues
  
  • Patients have a 70% reduced opening
  • Characteristic furrows radiating from the mouth produce a “purse string” appearance
• Xerostomia is frequently encountered
• A mild variant of systemic sclerosis exists:
  
  • Termed localized scleroderma
    
    • Affects a solitary patch of skin
    • Lesions look like scars, so the name en coup de sabre (“strike of the sword”) is used to describe them – You don’t need to know this again
    • Condition is typically purely cosmetic
• Management is difficult
• Effectiveness of treatment is difficult to assess because the condition waxes & wanes
• Corticosteroids are of little benefit
• Dental appliances are used to combat microstomia
• Prognosis is poor unless patients only have limited cutaneous scleroderma
• 10 year survival is approximately 60%
• Approaches 90% for those with limited cutaneous scleroderma

Question 152

For which type of syndrome are Osteomas very common with?

Answer 152

Gardner Syndrome

Question 153

Answer 153

Osteoid osteoma (nocturnal pain)

Question 154

What is the most common mesenchymal (non-epithelial, so soft tissue) neoplasm?

Answer 154

Lipoma, all though it is more common on the trunk.

Question 155

If a patient age 20 or younger has an OKC, he or she should be further evaluated to rule out which syndrome?

Answer 155

Gorlin syndrome

Question 156

During what age is Fibrous Dysplasia - Monostotic usually diagnosed?

Answer 156

In teenage years.

Question 157

What is a Rhabdomyoma, and where do the extracardiac rhabdoymyomas most commonly occur in adults?

Answer 157

It is a benign neoplasm of skeletal muscle, and they most commonly occur in adult men (70%) in the pharynx and floor of mouth.

Question 158

What is glossodynia?

Answer 158

Painful tongue

Question 159

What is the most common form of Neurofibromatosis?

Answer 159

Type 1 (NF1)

Question 160

Answer 160

Central giant cell granuloma, OKC, ameloblastoma. (if impacted tooth include dentigerous cyst)

Question 161

What would Gorlin Syndrome look like on an XRAY for CPC?

Answer 161

Unlilocular (most likely) OKCs in various locations, both mandibular and maxillary, all over, and in Kids I think, he would want us list the 3 or 4 other things associated with Gorlin syndrome (rib anomalies, calcification of falx cerebri, OKCs, basal cell carcinoma), hence the name nevoid basal cell carcinoma.

Question 162

What did WHO reclassify the odontogenic keratocyst as?

Answer 162

Keratocystic Odontogenic Tumor (KOT)

Question 163

What is the average age for a buccal bifurcation cyst?

Answer 163

Children, age 10

Question 164

Answer 164

Inflammatory Fibrous Hyperplasia

• Tumor-like hyperplasia of fibrous connective tissue
• Epulis Fissuratum is the name given for inflammatory fibrous hyperplasia in association with the flange of an ill-fitting denture
• Usually develops on the facial aspect of the alveolar ridge
• Treatment is surgical removal and remake the denture

Question 165

Answer 165

Dentigerous Cyst

• Cyst that originates by separation of the follicle from around the crown of an unerupted tooth
  
  • Apparently develops by accumulation of fluid between the reduced enamel epithelium and the tooth crown – If the space is more than 3 mm, than there is fluid in there and the cyst will grow. The lining is stratified squamous epithelium.
• Most common developmental cyst
• Encloses the crown of the unerupted tooth and is attached at the CEJ
• Most often involve mandibular 3rd molars
  
  • Rarely involve unerupted deciduous teeth
• Most frequently found in patients ages 10-30
• Completely asymptomatic & discovered on routine XRAY
• Usually do not, but can grow to considerable size & expand bone, cause facial asymmetry, etc.
• That’s why they must be removed (that and to rule out other cysts like OKC)
• Via XRAY:
  
  • Unilocular radiolucency associated with crown of unerupted tooth
  • Well-defined and usually sclerotic border
• Treatment is enucleation of the cyst together with the unerupted tooth
• Large examples may be treated by marsupialization
• Prognosis is excellent and recurrence is rare

Question 166

If lymphoid hyperplasia appears on the posterior lateral tongue, it is usually unilateral. True or False?

Answer 166

False, usually bilateral.

Question 167

During what ages does cherubism take place?

Answer 167

Between 2-5. The appearance is virtually diagnostic due to the bilateral symmetrical location in a young person – know this. Multilocular radiolucencies involving multiple quadrants of the mouth.

Question 168

Answer 168

Pyogenic Granuloma

• Not a true granuloma, but is a reactive lesion to local irritation or trauma (poor oral hygiene)
• May exhibit rapid growth
• Striking predilection for gingiva (75%), followed by lips, tongue and buccal mucosa
• Most common in children and young adults
• Smooth or lobulated mass that is typically pedunculated
• Surface is characteristically ulcerated and lesion bleeds easily
• Young lesions appear red; older lesions are more collagenized and pink
• Female predilection
  
  • Frequently occurs in pregnant women, most commonly in the 1st trimester
  • a.k.a pregnancy tumor or granuloma gravidarum
    
    • Some regress after pregnancy
• Treatment is surgical excision
• For gingival lesions, the excision should extend to periosteum and adjacent teeth scaled
• Lesions occasionally recur
• For lesions developing during pregnancy, defer treatment unless functional or esthetic problems develop

Question 169

What is a Leiomyoma, and where are the three most common locations?

Answer 169

It is a benign tumor of smooth muscle, and is most common in

1. Uterus
2. GI tractt
3. Skin

Question 170

Plummer-Vinson is considered to be a pre-malignant syndrome. True or false?

Answer 170

True

Question 171

What are the characteristics of Metastatic Tumors to the Jaws?

Answer 171

• Metastatic carcinoma is the most common form of cancer involving bone
• Most common origin of gnathic metastases:
  
  • Breast - F
  • Lung – M + F
  • Thyroid
  • Prostate - M
  • Kidney
• Metastatic Tumors to the Jaws
  
  • Affects older patients
  • Most common sites:
    
    • Vertebrae
    • Ribs
    • Pelvis
    • Skull
  • 80% of jaw mets occur in the mandible
• Symptoms:
  
  • Pain
  • Swelling
  • Loosening of teeth
  • Paresthesia
• Mets to the mandible with involvement of the inferior alveolar nerve occasionally produces a distinctive pattern of anesthesia, termed numb-chin syndrome
  
  • Unexplained loss of sensation in the lower lip and chin
• Metastases may be discovered in a non-healing extraction site
• XRAY shows features typical of a malignancy:
  
  • Ill-defined borders (“moth-eaten”)
  • Widening of the PDL ligament
  • Some may stimulate new bone formation, resulting in a mixed RL-RO
• Prognosis is poor (Stage IV disease)
• 5-year survival is exceedingly rare
• Pts typically succumb to cancer within a year

Question 172

Answer 172

Nevoid Basal Cell Carcinoma Syndrome

• A system complex characterized by: - Know this list below
  
  • Multiple basal cell carcinomas (BCCAs)
  • Odontogenic keratocysts
  • Calcification of the falx cerebri
  • Rib anomalies (splayed or bifid ribs)
  • Many others

Question 173

How may a peripheral giant cell granuloma differ from a pyogenic granuloma?

Answer 173

PGCG has usually a bluish hint.

Question 174

What is a “driven-snow” pattern on an XRAY associated with?

Answer 174

Calcifying Epithelial Odontogenic Tumor

Question 175

Where do 50% of cases occur with a peripheral ossifying fibroma?

Answer 175

In the incisor-cuspid region

Question 176

What is the difference between Antoni A and Antoni B in regards to Schwannoma histopathology?

Answer 176

Antoni A is organized, schwann cells form a palisaded arrangement around eosinophilic areas known as Verocay bodies.

Antoni B is disorganized.

Question 177

Answer 177

Ameloblastic Fibroma

• True mixed tumor - both epithelial and mesenchymal tissues are neoplastic – everything up to this point has been epithelial
• Occur in younger patients – usually before age 20
• Small tumors are asymptomatic; larger ones cause painless swelling
• 70% are located in the posterior mandible
• Via XRAY:
  
  • Can be unilocular or multilocular
  • Margins are well-defined and sclerotic
  • 75% are associated with an unerupted tooth
• Conservative initial therapy seems appropriate; recurrences require more aggressive surgery
• 50% of the ameloblastic fibrosarcomas develop in the setting of a recurrent ameloblastic fibroma

Question 178

What are the three main characteristics of MEN, type 2B?

Answer 178

1. Adrenal gland tumors (pheochromocytoma) (50%)
2. Thyroid tumors (medullary carcinoma) (90%)
3. Mucosal neuromas with a predilection for the oral cavity

Question 179

What pathology is associated with a fine “ground-glass” opacification?

Answer 179

Fibrous Dysplasia - Monostotic. Results from superimposition of poorly calcified bone in a disorganized fashion

Question 180

Answer 180

Gingival cyst of the adult

Question 181

Which impacted teeth are calcifying epithelial odontogenic tumors most often associated with?

Answer 181

Mandibular Molars

Question 182

Answer 182

OKC?

Question 183

What is glossopyrosis?

Answer 183

Burning of tongue (associated with anemia)

Question 184

Answer 184

Buccal Bifurcation Cyst

• XRAY features:
• Well-circumscribed, unilocular radiolucency involving the buccal bifurcation and root
• Average size is 1.5 cm
• An occlusal XRAY can demonstrate buccal location
• The root apices are characteristically tipped toward the lingual mandibular cortex
• This will be partially erupted, if it was fully impacted, we would think dentigerous cyst.

Question 185

Which virus is responsible for Burkitt’s Lymphoma?

Answer 185

EBV

Question 186

Answer 186

Sturge-Weber Angiomatosis

• Rare, nonhereditary developmental condition
• Caused by the persistence of a vascular plexus around the cephalic portion of the neural tube
• Patients are born with a dermal capillary vascular malformation known as port wine stain or nevus flammeus – Not all patients with a port wine stain have Sturge-Weber
• Has unilateral distribution along one or more segments of the trigeminal nerve
• Not all patients with a port wine stain have Sturge-Weber angiomatosis
• Patients with involvement along the distribution of the ophthalmic branch are at risk for full condition
• Sturge-Weber Angiomatosis
• In addition to nevus flammeus, patients have leptomeningeal angiomas
• Overlie the ipsilateral cerebral cortex
• Typically associated with a convulsive disorder – Might be on medication that causes gingival hyperplasia
• May result in mental retardation or contralateral hemiplagia (one-sided paralysis)
• Intraoral involvement is common and results in hypervascular changes to the ipsilateral mucosa
• Lesion may hemorrhage when manipulated

Question 187

Answer 187

Osteoblastoma

• Rare, especially in jaws
• Of lesions in the jaws:
• Md>Mx
• Posterior>Anterior
• 85% occur before age 30
• F>M
• Osteoblastomas are between 2-4 cm
  
  • Pain, tenderness, and swelling are important and common presenting features
  • Pain is NOT relieved by aspirin – because they are too big
• Via XRAY:
  
  • Well-defined or ill-defined – looks similar to cemento-osseus dysplasia, but if it is not in a tooth-bearing area, it cannot be cemento-osseus dysplasia, and will most likely be osteoblastoma or osteoid osteoma.
  • Radiolucent with varying degrees of central RO

Question 188

Where is bone pain most common for multiple myeloma patients?

Answer 188

In the lumbar spine.

Question 189

Which type of leukemia is one of the more common childhood malignancies? And which type of leukemia is the most common overall that occurs in adulthood?

Answer 189

Acute Lymphoblastic Leukemia (ALL). Chronic Lymphocytic Leukemia. (CLL)

Question 190

Where do most adenomatoid odontogenic tumors occur in the mouth?

Answer 190

In the anterior jaw

Question 191

Answer 191

CEOT

Question 192

With which disease are mandibular lesions “scooped out” because the superficial alveolar bone is destroyed?

Answer 192

Langerhans Cell Histiocytosis

Question 193

What is the difference between sarcoma and carcinoma?

Answer 193

• Sarcomas develop in your mesodermal tissue and are mesenchymal in origin. They include cancers of the Bone, Muscle, Fat, Nerves, Cartilage, Fibrous tissue, such as ligaments and connect tissue, Blood cancers, Leukemia, Lymphoma, Myeloma. Comprise only 1% of cancers. These grow in ball-like masses and spread through satellite nodules.
• Carcinomas originate in your epithelial tissue, such as the lining of your breast, lung, colon, prostate, kidney, thyroid, stomach, etc. Comprise more than 90% of cancers worldwide, and spread throughout body through blood and lymph.

Question 194

Is an odontoma a true neoplasm or a developmental anomaly?

Answer 194

Developmental anomaly (hamartomas)

Question 195

In what region of the mouth do gingival cysts of the adult usually appear?

Answer 195

Mandibular canine/premolar area

Question 196

Are the teeth associated with traumatic bone cysts vital?

Answer 196

Yes, they are vital

Question 197

What is the name for te malignant counterpart of the Ameloblastic Fibroma?

Answer 197

Ameloblastic Fibrosarcoma

Question 198

What does the plexiform variant of neurofibromatosis feel like and what does it mean if you have it?

Answer 198

It feels like a bag of worms, and if you have it, you have the disease, pathognomonic.

Question 199

What is the first sign of Systemic Sclerosis?

Answer 199

Raynaud’s Phenomenon, which is vasoconstrictive event triggered by emotional distress or exposure to cold – the tips of the fingers go white and you get that numb sensation, but this isn’t specific to systemic sclerosis. You also get short clawlike fingers with this.

Question 200

What are Bence Jones proteins associated with?

Answer 200

• Multiple Myeloma. Renal failure may be a presenting sign because the kidneys become overburdened with the excess circulating proteins
• Up to 50% of patients will multiple myeloma will have these proteins in the urine
• Termed Bence Jones proteins

Question 201

There is a strong association between Focal Osteoporotic Marrow Defect and hematologic disorders. True or False?

Answer 201

False. No association.

Question 202

Answer 202

Paget’s Disease of Bone

Question 203

Answer 203

Eruption Cyst

• Soft tissue analogue of the dentigerous cyst – know this
  
  • Develops as a result of separation of the dental follicle from around the crown of an erupting tooth
  • Occurs within the soft tissues overlying the alveolar bone
• Appears as a soft, translucent swelling in the gingival mucosa overlying the crown of an erupting tooth
• Usually seen in children younger than 10
• Surface trauma may result blood to accumulate in the cystic fluid, which imparts a blue or purple color
  
  • Called eruption hematomas
• Treatment may not be required
  
  • Cyst usually ruptures spontaneously
  • If this doesn’t occur, excision of the roof of the cyst permits eruption of the tooth

Question 204

Sturge-Weber Angiomatosis is Hereditary. True or False?

Answer 204

False.

Question 205

Answer 205

1. Mixed so there is 4 things:
   
   1. COC
   2. CEOT
   3. AOT
   4. AFO

Question 206

For what pathology are the terms “soap bubble” and “honeycombed” used?

Answer 206

Ameloblastoma.

• “Soap bubble” – when the RL loculations are large
• “Honeycombed” – when the loculations are small
• **Buccal and lingual cortical expansion is frequently present**
• Resorption of roots is common

Question 207

What are the three most common origins of gnathic metastases?

Answer 207

1. Breast
2. Lung (male and female)
3. Prostate

Question 208

Which pathology has the “starry-sky” pattern for histopathology, due to macrophages within tumor tissue?

Answer 208

Burkitt’s Lymphoma

Question 209

Where are the majority of traumatic bone cysts found? Where are they most commonly found in the mouth?

Answer 209

In long bones. Almost exclusively in the mandible.

Question 210

Vitamin C name and function?

Answer 210

Vitamin C (ascorbic acid): necessary for the proper synthesis of collagen. Scurvy.

Question 211

What are the two pathologies associated with supernumerary teeth?

Answer 211

Cleidocranial Dysplasia and Gardner Syndrome

Question 212

Traumatic Bone Cysts and Aneurysmal Bone Cysts are both “true” cysts with epithelial linings. True or False?

Answer 212

False. Neither of them are.

Question 213

Answer 213

Mycosis Fungoides

• So-named because it clinically resembles a fungal infection
• It is actually a T-cell lymphoma
• Most common cutaneous lymphoma
• Avg age = 60; M:F 2:1
• Progresses through three stages:
  
  • Eczematous
    
    • Often mistaken for psoriasis; well-demarcated, scaly, itchy, erythematous plaques
  • Plaque
    
    • Elevated red lesions
  • Tumor
    
    • Distinct papules and nodules; visceral involvement
• Sézary syndrome is an aggressive expression of mycosis fungoides that represents dermatopathic T-cell leukemia – Know for test
  
  • This condition runs a fulminant course and results in the patient’s death within 3 years
• Histopathologic features:
  
  • Atypical lymphocytic cells infiltrate the surface epithelium. (Epidermotropism is a propensity to invade the epidermis of the skin)
  • These atypical lymphocytes are termed mycosis cells or Sézary cells
  • The atypical lymphocytes form small intraepithelial aggregates termed Pautrier’s microabscesses
• Topical therapy is initiated first but will ultimately fail
• Aggressive chemotherapy is usually necessary, especially with visceral involvement
• The disease is not curable, but is usually slowly progressive
• Median survival time is 10 years

Once the disease progresses beyond cutaneous involvement, the patient usually dies within a year from organ failure or sepsis

Question 214

What is another name for Osteopetrosis?

Answer 214

Marble Bone Disease

Question 215

What are the characteristics of a benign bone neoplasia?

Answer 215

• Asymptomatic
• Grows slowly and by expansion: displaces teeth and expands the cortex
• Symmetrical -Usually corticated and well-defined border, which means that the bone resorption and growth is going slow enough to capture the transition.
• Does not metastasize

Question 216

What is koilonychia and what is it associated with?

Answer 216

It is spoon-shaped and brittle nails, and is associated with Plummer-Vinson syndrome.

Question 217

Peuts-Jeghers Syndrome intestinal polyps will become malignat. True or False?

Answer 217

False. But the ones with Gardner Syndrome will, so you would remove the colon.

Question 218

During what decades of life are ameloblastomas most common?

Answer 218

Between 3rd to 7th decade

Question 219

Answer 219

Ameloblastic fibroma

Question 220

Answer 220

Graft-Versus-Host Disease

• Occurs in recipients of allogeneic bone marrow transplantations
• If the match isn’t exact, the engrafted cells recognize they aren’t in their own environment
  
  • When this happens, the engrafted cells attack what they perceive as a foreign body, resulting in GVHD
• Systemic signs are varied, depending on the organ system involved and the severity of disease
• More mild disease is seen in patients who:
  
  • Have a better histocompatibility match
  • Are young
  • Are female
  • Have received cord blood
• Disease may be acute or chronic
• Looks a lot like lichen planus and others, but this will show in multiple areas of the body, several, and they have history of leukemia and transplant.
• Acute GVHD:
  
  • Observed within about a month
  • Arbitrarily defined as occurring within 100 days after the procedure
  • Affects 50% of bone marrow transplant (BMT) patients
  • Skin lesions range from a mild rash to diffuse and severe sloughing that resembles toxic epidermal necrolysis
  • Patients may also have diarrhea, nausea, vomiting, abdominal pain, and liver dysfunction
  • Ulcerations may develop related to the chemotherapy and neutropenic state during the first 2 weeks after BMT; those that persist represent acute GVHD
• Chronic GVHD:
  
  • May represent a continuation of acute GVHD or it may develop later than 100 days after BMT
  • Can appear up to several years after the procedure
  • Develops in up to 70% of BMT patients
  • Mimics a variety of autoimmune conditions, such as systemic lupus erythematous or Sjögren syndrome
• Oral manifestations:
  
  • Of those with acute GVHD, 50% have oral involvement
  • Of those with chronic GVHD, more than 80% have oral involvement
    
    • Most patients have a fine, reticular network of white striae which is indistinguishable from lichen planus
    • Atrophy or ulceration may also occur
    • Xerostomia is a common complaint
• Treatment:
• Primary strategy is to prevent its occurrence
• If GVHD develops, immunosuppressive drug doses are increased
• Topical corticosteroids may be used for focal oral ulcerations
• Prognosis of patients with GVHD depends on the extent to which the condition progresses and whether it can be controlled
• More than 70% survive mild GVHD
• 15% survive severe GVHD

Question 221

Answer 221

Central Giant Cell Granuloma

Question 222

Answer 222

Leukemia

• Represents several types of malignancies of hematopoietic stem cell derivation
  
  • Begins as the malignant transformation of a stem cell, which proliferates in the bone marrow and overflows into the peripheral blood
• Leukemias are classified according to their histogenesis and clinical behavior:
  
  • Myeloid vs lymphoid
  • Acute vs chronic
• Examples: - Know these four
  
  • Acute myeloid leukemia
  • Chronic myeloid leukemia
  • Acute lymphoblastic leukemia
  • Chronic lymphocytic leukemia
• Acute leukemias run an aggressive course and will lead to the death of the patient in a few months if untreated
• Chronic leukemias run a more indolent course, though patients eventually succumb to the disease
• Environmental and genetic factors contribute to the development of leukemia
• Certain leukemias show specific chromosomal abnormalities:
  
  • Chronic myeloid leukemia (CML) has a translocation of the chromosomal material between the long arms of chromosomes 9 & 22
    
    • This is termed the Philadelphia chromosome
• Some of the environmental factors which may lead to leukemia:
  
  • Exposure to pesticides and/or benzene
  • Ionizing radiation
• Viruses, such as human T-cell leukemia/lymphoma virus type 1 (HTLV-1)
  
  • Transmitted by blood
• Acute lymphoblastic leukemia (ALL) is one of the more common childhood malignancies – Know this
• Chronic lymphocytic leukemia is the most common type; it occurs in adulthood – Know this
• Clinical signs and symptoms are related to the reduction in numbers of normal RBCs and WBCs
  
  • Decreased oxygen-carrying capacity of the blood leads to fatigue
  • Patients may experience easy bruising due to lack of platelets
• The leukemic cells may infiltrate the oral soft tissues to produce a boggy, non-tender swelling – Can look like drug-induced gingival hyperplasia, but when you palpate it, it is fairly soft. The classica gingival leukemia looks dark and black and kind of like melanoma, but it is so diffuse so it is easier to differentiate between melanoma and Kaposi’s sarcoma. And with the picture below, you would think salivary gland neoplasm until you palpate, and if it is soft, most likely not neoplasm, and this picture is one of Leukemia.
  
  • This is termed granulocytic sarcoma; historically
• If leukemia is suspected, referral to the appropriate physician is mandatory
• 80% of children survive ALL
• Patients with AML have a 40% survival if they are younger than 60; 10% if not
• CML’s 5-year survival is 80%, but once blast transformation occurs, the patient will usually die within 6 months
• CLL is incurable; patients typically survive 10 years

Question 223

Answer 223

CCLE

• Few or no systemic signs
• Lesions are limited to skin or mucosal surfaces
• Skin lesions present as discoid lupus erythematous
  
  • Begin as scaly, erythematous patches on sun-exposed skin
  • Especially affect the head and neck
• Lesions may heal and recur
• Healing leads to cutaneous atrophy, scarring, and altered pigmentation
• Oral lesions, if present with the skin lesions, resemble lichen planus

Question 224

Answer 224

Osteosarcoma

• Mesenchymal malignancy
• Cells have the ability to produce osteoid or immature bone
• Excluding hematopoietic neoplasms (leukemias, lymphoma, etc), it is the most common type of malignancy to originate within bone
• May be intramedullary, juxtacortical, or extraskeletal
• Extragnathic osteosarcomas have a bimodal age distribution:
  
  • Between 10-20 – More common – know this, know the ones that are more common in children, and when it is, it is not found much in the jaw. Look below.
  • After 50
• Most common place for osteosarcomas to arise in young patients:
  
  • Distal femur
  • Proximal tibia
• Osteosarcomas of the jaws comprise 7% of all osteosarcomas
  
  • Occur around age 33
    
    • This is 10-15 years older than osteosarcoma of the long bones
• Mx = md
  
  • Mandibular tumors are more likely to be in the posterior jaws
  • Maxillary lesions are more common inferiorly rather than superiorly
    
    • Inferiorly = alveolar ridge, sinus floor, palate
    • Superiorly = zygoma, orbital rim
• Swelling and pain are common symptoms
• XRAY features vary:
  
  • May be RO, RL with RL border, mixed, or RL
  • The peripheral border is ill-defined
  • Extensive osteosarcomas may only show minimal and subtle changes
  • Root resorption can occur; which is termed spiking resorption – tapered narrowing of root
  • **Classic is sunburst appearance – found only in 25% and due to bony projections on the surface of the lesion** - If you see sunburst, you are thinking bone cancer, but not all bone cancer has sunburst appearance.
    
    • Best appreciated with an occlusal XRAY
  • A triangular elevation of periosteum termed Codman’s triangle can occur – Know this
  • An important, early finding is a symmetrical widening of the PDL space around one or more teeth
    
    • The PDL widening is due to tumor infiltration
      
      • Not specific for osteosarcoma; seen in other malignancies
• Though periapical, occlusal, and pano XRAYs can lead to dx, CT scans are used to determine extent of tumor most accurately
• May have a variety of histopathologic subtypes: - We don’t need to know these subtypes
  
  • Osteoblastic
  • Chondroblastic
  • Fibroblastic
  • Etc..
• Most important prognostic indicator is ability to achieve initial complete surgical removal
  
  • Therefore, in general, md lesions have a better prognosis
• Long bone treatment:
  
  • Preoperative chemotherapy
  • Radical surgical excision
  • +/- Postoperative chemotherapy
  • Survival rates approach 80%
• Jaw lesions have a less tendency to metastasize than long bone counterparts; lung and brain are the most commonly affected sites
  
  • Prognosis for jaw lesions is between 30-70%, depending on the study
    
    • Can approach 80% using initial radical surgery
• Juxtacortical Osteosarcoma
  
  • AKA Peripheral osteosarcoma
  • Juxtacortical lesions originate on the cortex of the bone and gown outward
  • Most commonly found in the long bones
    
    • Two types:
      
      • Parosteal
      • Periosteal

Question 225

What is the Central Giant Cell Granuloma histopathology identical to?

Answer 225

Identical to a brown tumor of hyperparathyroidism and lesions of cherubism.

Question 226

Answer 226

NK/T-cell Lymphoma

• Also known as angiocentric T-cell lymphoma (angiocentric because we are occluding vessels and causing hypoxia to certain tissues)
• Typically found in adults
• A rare process that is characterized clinically by an aggressive, nonrelenting destruction of the midline structures of the palate and nasal process
  
  • Swelling of the palate may precede the formation of a deep, necrotic ulceration
  • Ulceration is usually found in the midline
  • The ulceration enlarges and destroys the palatal tissues
• Without treatment, the patient will succumb to secondary infection, massive hemorrhage, or infiltration of vital structures
• Lesions typically respond to radiation therapy
• 5-year survival is 85%
• Patients with disseminated disease require chemotherapy and have a 40% 5-year survival

Question 227

Answer 227

Differ: dentigours cyst, okc, ameloblastoma central giant cell,

Question 228

What causes Sturge-Weber Angiomatosis?

Answer 228

Caused by the persistence of a vascular plexu around the cephalic portion of the neural tube.

Question 229

What type of collagen maturation has the defect with Osteogenesis Imperfecta?

Answer 229

Type I collagen

Question 230

Answer 230

Florid Cemento-Osseous Dysplasia

• Multiple focal involvement not limited to the anterior mandible
• Pts may just have lesions in the post jaws, but many patients have lesions throughout
• 90% are female; 90% are African American
• Occurs in middle-aged or older adults
• Marked tendency to be bilateral and symmetrical
• May be completely asymptomatic
• Via XRAY, lesions demonstrate an identical pattern of maturation noted in the other two forms:
• Initially, lesions are predominantly radiolucent
  
  • Over time become mixed RL-RO
  • End-stage lesions are predominantly radiopaque with a thin peripheral RL rim
  • Involvement is unrelated to presence or absence of teeth

Question 231

Answer 231

Idiopathic Osteosclerosis

Question 232

Osteoblastomas and and Osteoid Osteomas both produce prostaglandins, and are both relieved by aspirin. True or False?

Answer 232

False. Osteoblastomas are too big to be relieved by aspirin.

Question 233

What are the characteristics of Hypothyroidism?

Answer 233

• Decreased levels of thyroid hormone
• Cretinism: when occurs during infancy
• Myxedema: when in adulthood

Question 234

Answer 234

Fibrous Dysplasia - Polyostotic

• Involvement of two or more bones; can involve up to 75% of skeleton
• If jaw is involved, facial asymmetry may result
• Jaffe-Lichtenstein syndrome:
  
  • Polyostotic fibrous dysplasia
  • Café au lait spots (coast of Maine) – also involved with neurofibromatosis
• McCune-Albright Syndrome:
  
  • Polyostotic fibrous dysplasia
  • Café au lait spots (coast of Maine)
  • Multiple endocrinopathies
    
    • Sexual precocity (early onset puberty), pituitary adenoma, and/or hyperthyroidism
      
      • Sexual precocity is the most common
        
        • Menstrual bleeding, breast development, and pubic hair appear in females within first few years of life
• Clinical management is a major problem
  
  • Smaller lesions of the mandible may be resected
  • Diffuse and large nature of most lesions precludes this option
• Disease tends to stabilize and stop growing at skeletal maturity
• May reduce surgically; up to 50% recur

Radiation therapy is contraindicated

Question 235

What type of tumor is congenital epulis?

Answer 235

Granular Cell Tumor

Question 236

Answer 236

1. Desquamtive gingivitis:
   
   1. Pemphigus
   2. Mucus membrane pemphigo
   3. Lichen Planus

Question 237

Answer 237

Adenomatoid Odontogenic Tumor

• Occurs in young patients, ages 10-20
• Very uncommon in patients over the age of 30
  
  • *Striking tendency to occur in the anterior jaws
  • *Mx>Md
  • *F:M 2:1
• Most are relatively small; may also rarely occur peripherally
• **Asymptomatic and discovered during XRAY to determine why a tooth hasn’t erupted**
• *In 75% of cases, the tumor is a circumscribed, unilocular RL involving the crown of an unerupted tooth*
  
  • Most often a canine tooth – helps distinguish it from the other differentials like dentigerous cyst.
• RL often extends apically past the CEJ; this can help differentiate from dentigerous cyst
• Lesion may contain fine, snowflake calcifications via XRAY – can also help differentiate from dentigerous cyst – so mostly radiolucent, but some calcifications, so the differentials for this combination are adenomatoid odontogenic tumor, calcifying odontogenic cyst, and calcifying epithelial odontogenic tumor. (AFO is also differentials, next page down, also, desmoplastic amelofibroma)
• If around impacted canine, it would be AOT
• Unilocular radiolucency in between canine and premolar roots, lateral periodontal cyst.
• If it is a 10 year old child (you can tell from teeth), posterior mandible, impacted tooth, mixed radiolucency and radiopaque, then AFO is the answer.
• Lesion may also develop as a well-defined, unilocular RL between roots
• Tumor has a thick, fibrous capsule that makes enucleation easy
• Recurrence doesn’t occur; lesion is completely benign & aggressive behavior has not been documented
• Prognosis is good

Question 238

What is TTP and ITP?

Answer 238

• Thrombotic Thrombocytopenic Purpura and Idiopathic Thrombocytopenic Purpura.
• ITP occurs in childhood, classically after a viral infection
• 90% of cases resolve in 6 months
• TTP is a more serious disorder of coagulation and is probably due to endothelial damage

Question 239

What is intussusception and what syndrome is it associated with?

Answer 239

It is “Telescoping” of a proximal segment of the bowel into the distal portion, and is associated with intestinal obstruction in Peutz-Jeghers syndrome.

Question 240

Answer 240

Langerhans Cell Histiocytosis

• Langerhans cells are dendritic mononuclear cells normally found in the epidermis, mucosa, lymph nodes, and bone marrow
• They are antigen presenting cells
• There is a spectrum of disease: - He might ask us about these, but will let us know
  
  • Eosinophilic granuloma of bone: One or multiple lesions; no visceral involvement
  • Acute disseminated histiocytosis – Disease involves bone, skin, and viscera; more prominent involvement; mostly in infants (Letterer-Siwe disease)
  • Chronic disseminated histiocytosis – Disease involves bone, skin, and viscera (Hand-Schüller-Christian disease)
• More than half of patients are younger than 15 years old
• Bone lesions are the most common presentation
• Lesions are found most commonly in the:
  
  • Skull
  • Ribs
  • Vertebrae
  • Mandible
• Jaws are affected in 15% of cases
• Pain and tenderness are the most common accompanying symptoms
• XRAY:
  
  • Sharply demarcated (“punched-out”) radiolucencies
  • Occasionally may be ill-defined
  • Mandibular lesions appear “scooped out” because the superficial alveolar bone is destroyed
  • Extensive alveolar bone involvement resembles severe periodontitis clinically and radiographically the teeth look as though they are “floating in air”
• Histopathologic features:
  
  • Identification of a lesional Langerhans cell is necessary to confirm diagnosis
  • Ultrastructurally, Langerhans cells contain rod-shaped cytoplasmic structures known as Birbeck granules, which differentiated them from other APCs – You have to do electron Microscopy to find this out
• Bone lesions are treated by curettage or low-dose radiation

The prognosis of disease limited to bone is good; those with visceral and cutaneous involvement have a poor prognosis

Question 241

Vitamin A name and function?

Answer 241

Vitamin A (retinol): essential for vision. May lead to blindness.

Question 242

What type of disorder is Sturge-Weber Angiomatosis associated with?

Answer 242

• Typically associated with a convulsive disorder – Might be on medication that causes gingival hyperplasia
• May result in mental retardation or contralateral hemiplagia (one-sided paralysis)

Question 243

Where does a peripheral giant cell granuloma exclusively occur?

Answer 243

On the gingiva or edentulous alveolar ridge.

Question 244

What is the recurrence rate for odontogenic keratocysts?

Answer 244

30%, they come back more often, unlike the dentigerous cyst.

Question 245

Answer 245

Peripheral Ameloblastoma

• Painless, nonulcerated, sessile gingival lesion
• Clinically interpreted to be one of the 3Ps – bump on the gum
• Avg age is 50
• Found most commonly on the posterior gingival mucosa, md>mx
• Unlike intraosseous ameloblastomas, the peripheral variant has innocuous clinical behavior
  
  • 15% recurrence; further excision has close to 0% recurrence

Question 246

Answer 246

Anemia

• General term for a decrease in volume of RBCs or in the concentration of hemoglobin
  
  • Hematocrit = volume of RBCs
• Anemia is often a sign of an underlying disease, such as:
  
  • Renal failure
  • Liver disease
  • Chronic inflammatory conditions
  • Malignancies
  • Vitamin or mineral deficiencies
• General symptoms are related to the reduced oxygen-carrying capacity of the blood:
  
  • Tiredness
  • Headache
  • Fainting/feeling lightheaded
  • Pallor
• Oral symptoms: mucosa may show pallor or the patient may have a bald tongue (tongue atropy), depending on the type of anemia, as well as burning (glossopyrosis) and pain (glossodynia) localize to the tongue. – Know these definitions
• Treatment depends on correcting the underlying condition; refer to physician

Question 247

What are the four characteristics that offer a more mild disease version of Graft-Versus-Host Disease?

Answer 247

More mild disease is seen in patients who:

• Have a better histocompatibility match
• Are young
• Are female
• Have received cord blood

Question 248

What disease is McCune-Albright Syndrome associated with, and what are its characteristics?

Answer 248

Fibrous Dysplasia - Polyostotic. Cafe au lait spots (Coasts of Maine), multiple endocrinopathies, sexual precocity (early onset puberty).

Question 249

What is the most common odontogenic tumor in general?

Answer 249

Odontoma

Question 250

What is Sezary syndrome?

Answer 250

An agressive expression of Mycosis Fungoides that represent dermatopathic T-cell leukemia.

Question 251

Answer 251

Buccal Bifurcation Cyst

• Inflammatory cyst with uncertain pathogenesis
• Characteristically develops on the buccal aspect of the mandibular first permanent molar
• Occurs in children, avg age of 10
• Patient may experience tenderness, swelling, or foul-tasting discharge
• Perio probing usually reveals pocket formation on the buccal aspect
• 1/3 have bilateral involvement
• XRAY features:
  
  • Well-circumscribed, unilocular radiolucency involving the buccal bifurcation and root
  • Average size is 1.5 cm
  • An occlusal XRAY can demonstrate buccal location
  • The root apices are characteristically tipped toward the lingual mandibular cortex
  • This will be partially erupted, if it was fully impacted, we would think dentigerous cyst.
• Treated by enucleation
• No need to extract the tooth
• Within one year of surgery, there is usually complete healing

Question 252

What is the highly characteristic skin lesion associated with erythema multiforme?

Answer 252

Concentric circular erythematous rings, bull-eye, target lesions.

Question 253

What are the two pathologies associated with a 30% recurrence rate?

Answer 253

Glandular Odontogenic Cyst and Odontogenic Keratocyst

Question 254

What pathology is the “sunburst” appearance associated with?

Answer 254

Osteosarcoma, but only with 25% of cases, and it is due to bony projections on the surface of the lesion. Sunburst = bone cancer, but not all has it

Question 255

What is another name for a schwannoma? and what are the main features?

Answer 255

Acoustic neuromas of vestibular nerve, meningiomas and ependymomas of the central nervous systems.

Question 256

What is a fibrous histiocytoma called when it is on the skin?

Answer 256

Dermatofibroma

Question 257

What is the treatment for SLE and CCLE?

Answer 257

• SLE:
  
  • Patients should avoid excessive sunlight
  • Mild disease is managed by NSAIDs and antimalarial drugs (such as hydroxychloroquine)
  • More severe cases are treated with systemic corticosteroids and other immunosuppressive agents
• CCLE:
  
  • Patients should avoid excessive sunlight
  • Treated effectively with topical corticosteroids

Question 258

What is the most common fibro-osseous lesion encountered in clinical practice?

Answer 258

Cemento-Osseous Dysplasia

Question 259

What are the three most commonly affected areas in primary amyloidosis?

Answer 259

1. Eyelid
2. Neck
3. Lips

Question 260

What is the “eyes upturned to heaven” appearance associated with?

Answer 260

Cherubism

Question 261

Answer 261

Ameloblastoma

• Most common clinically significant odontogenic tumor – because of its recurrence rate, but odontoma is the most common odontogenic tumor in general.
• Tumors of odontogenic epithelial origin
• Three different types – board question:
  
  • Conventional solid or multicystic – 85% - This is multilocular, has the worst prognosis
  • Unicystic – 15%
  • Peripheral – 1%
• Equal prevalence in the 3rd – 7th decade
• No sex predilection
• 85% occur in the mandible, usually the molar-ascending ramus area – board question
• Tumor is asymptomatic; smaller lesions are detected only during routine XRAY
• If there is a clinical sign, it’s most often a painless swelling
• If untreated, can grow to grotesque proportions – even then, pain & paresthesia are uncommon
• Ameloblastoma
• The typical radiographic feature is a multilocular radiolucency
  
  • The lesion is described in one of two ways:
    
    • “Soap bubble” – when the RL loculations are large
    • “Honeycombed” – when the loculations are small
    • **Buccal and lingual cortical expansion is frequently present**
  • Resorption of roots is common
  • An unerupted tooth may be associated with the defect
• Six patterns: - we do not need to know these patterns
  
  • Follicular – most common
  • Plexiform – 2nd most common
  • Acanthomatous – has keratin
  • Granular cell
  • Basal cell – very rare
  • Desmoplastic – see below
• The desmoplastic ameloblastoma does not share features with the other forms:
  
  • Predilection for the anterior maxilla
  • May be mixed radiolucent-radiopaque – similar to calcifying odontogenic cyst
    
    • Due to osseous metaplasia
• Histopathologic features:
• **Palisading**, hyperchromatic basal layer – odontogenic keratocyst also has hyperchromatic
• **Reverse polarity** - nucleus is on opposite side of where it usually is
• You also have a red, white, and blue layer sequence on histology
• The three differentials for a multilocular (really any locular) radiolucency around an impacted tooth are dentigerous cyst, odontogenic keratocyst, ameloblastoma, central giant cell granuloma. And if you see root resorption, you would think ameloblastoma. If it is unilocular, most likely a dentigerous cyst (especially if with a tooth), then OKC, then eruption cyst.
• And if it crosses the midline, then glandular odontogenic cyst, CGCG, OKC, and ameloblastoma are the biggest, but if tooth is involved, CGCG drops to bottom of list.
• Apical vacuolization
• Patients with conventional solid or multicystic ameloblastomas are treated in a variety of ways, from simple enucleation & curettage to en bloc resection
  
  • Ameloblastomas infiltrate between cancellous bone at lesion periphery
  • In early stages, bone resorption cannot be seen via radiograph
  • Curettage have recurrence rates anywhere from 50 – 90%
  • Recurrence takes years
• Marginal resection is the most widely used treatment; recurrence rate is 15%
• Some surgeons take 1.5 cm margins beyond what is visible radiographically

Question 262

What is another name for NK/T-cell Lymphoma?

Answer 262

Angiocentric T-cel Lymphoma (angio centric because we are occluding vessels and causing hypoxia to certain tissues.

Question 263

Answer 263

Plummer-Vinson Syndrome

• A system complex characterized by:
  
  • Iron-deficiency anemia
  • Glossitis
  • Dysphagia
• Incidence is declining, likely due to improved nutritional status
• Condition is significant in that it has been associated with a high frequency of both oral and esophageal squamous cell carcinoma
• It is considered a premalignant process (It is like leukoplakia, you have a premaligant condition and needs to be monitored over time)
• Most patients are women of Scandinavian or Northern European background between the ages of 30 & 50
• Patients complain of a burning sensation associated with the tongue and oral mucosa
• Angular cheilitis is often present
• Marked atrophy of the lingual papilla produces a smooth, red appearance of the dorsal tongue
• Patients have pain or difficulty upon swallowing
• Evaluation of the esophagus shows the presence of abnormal bands of tissues termed esophageal webs
• Another sign is an alteration of the growth pattern of the nails
  
  • Results in a spoon-shaped configuration (koilonychia)
  • Nails are also brittle
• Treatment is directed at correcting the iron-deficiency anemia by means of dietary iron supplementation
• Patients should be evaluated periodically for oral, pharyngeal, and esophageal carcinoma
• Anywhere from a 5 to 50% chance of developing an upper aerodigestive tract malignancy

Question 264

What is the most common difference between cementoblastomas and cemento-osseous dysplasia?

Answer 264

Cementoblastomas are usually fused to one or more tooth roots, and they are also radiopaque masses.

Question 265

What is the name of the dermal capillary vascular malformation associated with Sturge-Weber Angiomatosis? And not all patients have this malformation? True or False?

Answer 265

Known as Port Wine Stain or nervous flammeus, and True, not all patients have this.

Question 266

What is the soft tissue analog of the dentigerous cyst?

Answer 266

Eruption Cyst

Question 267

Ossifying Fibroma is not a true neoplasm. True or False?

Answer 267

False. It is, and has significant growth potential.

Question 268

What are the differentials for a radiolucency around an impacted tooth?

Answer 268

Dentigerous cyst, OKC, ameloblastoma, CGCG. And if you see root resorption, you would think ameloblastoma. If it is unilocular, most likely a dentigerous cyst (especially if with a tooth), then OKC, then eruption cyst. And if it crosses the midline, then glandular odontogenic cyst, CGCG, OKC, and ameloblastoma are the biggest, but if tooth is involved, CGCG drops to bottom of list.

Question 269

Which 1/3rd of the face does Paget’s Disease often cause enlargement in?

Answer 269

Middle 1/3rd of face, resulting in Lionlike facial deformity (Leontiasis Ossea)

Question 270

Pyogenic granuloma is not a true granuloma. True or False?

Answer 270

True, but is a reactive lesion to local irritation or trauma.

Question 271

Which pathology is associated with a RL defect that contains thin, wispy trabeculae of residual bone, which are often arranged at right angles to one another?

Answer 271

Odontogenic Myxoma (but he won’t ask us to identify on radiographs because too similar to others)

Question 272

What is and what are the two common locations of rhabdomyosarcoma?

Answer 272

Malignancy of skeletal muscles, and face and orbit are the two common locations.

Question 273

What is the process of a lesion turning into an odontoma? And what is the average age for all of these lesions?

Answer 273

An ameloblastic fibroma (AF) turns into an ameloblastic fibro-odontoma (AFO), which turns into an odontoma. Average age is young, 10-20.

Question 274

Where are the most common intraoral sites of neurofibromas?

Answer 274

Tongue and buccal mucosa

Question 275

Which pathology looks similar to Cemento-Osseous Dysplasia on an XRAY, but it not usually found in a tooth-bearing area, (where cemento-osseous dysplasia are only found)?

Answer 275

Osteoblastoma or Osteoid Osteoma

Question 276

Answer 276

Chondrosarcoma

• Malignant bone tumor of cartilage – Know this mainly, and that’s about it
• Up to 3% arise in the head and neck
• In the extragnathic bones, it is a tumor of adulthood – almost all patients are older than 50
• When occurring in the head and neck:
  
  • Mx:md 4:1
  • Avg age = 40
  • No sex or race predilection
• Painless mass or swelling is the most common presenting sign, which may be associated with separation or loosening of teeth
• Via XRAY, the tumor shows features suggestive of malignancy:
  
  • RL with poorly defined borders
  • The RL area contains variable amounts of RO foci
  • +/- root resorption, symmetrical PDL widening
• Chondrosarcomas often demonstrate extensive infiltration between osseous trabeculae of preexisting bone without causing significant resorption
  
  • Therefore, the tumor extent is difficult to determine radiographically
• The most important factor in prognosis is tumor location
  
  • This is because complete resection is the most effective treatment
• Radiation & chemo are less effective when compared to osteosarcoma
• Death is usually due to direct extension into vital structures rather than metastases
• Survival:
  
  • 5-year 85%
  • 10 year 70%
  • 15 year 50%
  • Recurrence is a late sequela and patients must be followed for life

Question 277

Answer 277

Nevoid Basal Cell Carcinoma Syndrome

• AKA Gorlin Syndrome – know this
• AD inherited
• Chromosome 9
  
  • PTCH gene
    
    • This gene abnormality has been detected in OKCs, which is why WHO wanted to change the name to represent a cystic neoplasm rather than a cyst
• Occurs in 1:60,000
• A system complex characterized by: - Know this list below
  
  • Multiple basal cell carcinomas (BCCAs)
  • Odontogenic keratocysts
  • Calcification of the falx cerebri
  • Rib anomalies (splayed or bifid ribs)
  • Many others
• The BCCAs appear around puberty and appear on skin not exposed to sunlight
• OKCs are one of the most constant features of the syndrome
  
  • Frequently multiple
• Patients with this syndrome usually do not have life threatening anomalies
• Prognosis depends on behavior of the skin tumors

Varying degrees of jaw deformity may result from operations for multiple OKCs

Question 278

Answer 278

OKC (know histo!) easy cheese

Question 279

Answer 279

Osteoid Osteomas

• Very rare in the jaws
• Pain is common
  
  • **Nocturnal**
  • **Relieved by aspirin** - Know these two
• Via XRAY:
  
  • Well-circumscribed
  • Radiolucent defect
  • Usually less than 1 cm (remember, less than 2 cm still indicates lesion is OO)
  • Small RO nidus may be present, resulting in a “target-like” appearance

Question 280

What are the characteristics of Fibrous Dysplasia?

Answer 280

• Tumorlike condition
  
  • Characterized by replacement of normal bone by fibrous connective tissue intermixed with bone
• Sporadic condition resulting from a postzygotic mutation – know this
  
  • Therefore, depending on when the mutation takes place, the process may involve:
    
    • One bone (monostotic)
    • Multiple bones (polyostotic)
    • Skin
    • Endocrine system
• Fibrous dysplasia
  
  • Types:
    
    • Monostotic
    • Polyostotic:
      
      • Jaffe-Lichtenstein Syndrome
      • McCune-Albright Syndrome

Question 281

Answer 281

Odontogenic Keratocyst

• Two most important things:
  
  • 30% recurrence rate –They can come back more often, unlike the dentigerous cyst.
  • Associated with a syndrome
• Arises from cell rests of the dental lamina
• WHO has reclassified this lesion as the “keratocystic odontogenic tumor” (KOT) – Boards might use this name now.
  
  • While this new terminology is being increasingly accepted and taught, most clinicians will refer to this lesion as an OKC
  • Your boards will probably say KOT, so you need to know both
• Most cases are diagnosed between ages 10-40
• Posterior mandible is the most common location
• **Tend to grow in an anterior-posterior direction**
• OKC is also associated with displacing a tooth way up near the orbit
• Doesn’t cause bone expansion
• Via XRAY:
  
  • Smaller lesions: well-defined, unilocular RL with corticated margins
  • Larger lesions: Multilocular
• An unerupted tooth is involved in 1/3 of cases
• Typically does not resorb roots of adjacent teeth
• Histopathology: - Know these characteristics
  
  • Thin, friable wall
  • Epithelial lining is a uniform 6-8 layers thick
  • Basal cell (dark blue) layer shows palisading (standing up like soldiers) & is hyperchromatic
  • Epithelium is surfaced by wavy or “corrugated” parakeratin (has nuclei)
  • May have small satellite cysts away from primary lesion – “daughter cysts” – another reason why recurrence rate is higher.
• Biopsy is required for diagnosis
• Most lesions are treated with enucleation and curettage
• Complete removal is often difficult due to the friable nature of the cyst wall
• Recurrence is 30% and may happen 10 or more years after surgery (long-term follow-up required) – This is the main one we worry about recurring.
• If a patient age 20 or younger has an OKC, he or she should be further evaluated to rule out Gorlin syndrome

Question 282

Why does microstomia occur with systemic sclerosis?

Answer 282

Microstomia occurs as a result of collagen deposition in the perioral tissues

Question 283

Biopsies are always needed with lichen planus. True or false?

Answer 283

• No biopsy is needed if the lesions are:
• Bilateral AND asymptomatic
• This is consistent with a clinical diagnosis of reticular oral lichen planus; no treatment is necessary

Question 284

What pathology is associated with the root apices characteristically tipped toward the lingual mandibular cortex on an XRAY?

Answer 284

Buccal bifurcation cyst. This will be partially erupted, if it was fully impacted, we would think dentigerous cyst.

Question 285

What are four of the main characteristics of Tuberous Sclerosis?

Answer 285

1. Mental retardation
2. Seizure disorders (meds can cause gingival overgrowth)
3. Angiofibromas of the skin
4. Characteristic skin lesions (Shagreen patches, Ash-leaf spots (this is a hypo-pigmentation, and it was on his boards, shagreen patches and café-ale are hyperpigmentation)

Question 286

What is the name of the neoplastic cells of Hodgkin’s Lymphoma? And what location is most common for Hodgkin’s Lymphoma?

Answer 286

Reed-Sternberg cells. 75% in the cervical and supraclavicular nodes.

Question 287

What percent of Focal Osteoporotic Marrow Defects are found in adult women?

Answer 287

75%, typically in posterior mandible

Question 288

What is the most common developmental cyst?

Answer 288

Dentigerous Cyst

Question 289

Where is the most common location in the mouth of the glandular odontogenic cyst?

Answer 289

Mandible, anterior jaw (crosses midline).

Question 290

What disease is Jaffe-Lichtenstein syndrome associated with, and what are its other characteristics?

Answer 290

Fibrous Dysplasia - Polyostotic. Cafe au lait spots (coast of maine), which is also involved with neurofibromatosis.

Question 291

What is the definition of Fibrous Dysplasia?

Answer 291

Sporadic condition resulting from a postzygotic mutation

Question 292

Answer 292

Neurofibroma

• Most common type of peripheral nerve neoplasm
• Can arise as solitary tumors or be a component of neurofibromatosis
• Most common in young adults and present as slowly-growing, soft, painless lesions
• Skin is the most common location
• Tongue and buccal mucosa are most common intraoral sites
• Treatment is surgical excision

Any patient with a lesion diagnosed as a neurofibroma must be evaluated for neurofibromatosis

Question 293

Answer 293

Ossifying Fibroma

• True neoplasm with significant growth potential
• Neoplasm composed of fibrous tissue that contains a variable mixture of bone & cementum
• Occur across a wide age range
  
  • F>M; md>mx
• Small lesions do not cause symptoms are detected via XRAY
• Large tumors result in a painless swelling
• XRAY features:
  
  • Well-defined
  • Typically unilocular
  • Can be completely radiolucent or, more commonly, mixed RL-RO
    
    • Depends on amount of calcified material;
  • Root divergence or resorption of roots can occur - while fibrous dysplasia and cemento-osseous dysplasia just goes around the roots.
• **Large ossifying fibromas of the mandible demonstrate a characteristic downward bowing of the inferior cortex of the mandible – know this
• Lesions can typically be enucleated easily
• Prognosis is very good
• Recurrence is very rare
• These tumors do not undergo malignant transformation

Question 294

What are the characteristics of Cemento-Osseous Dysplasias?

Answer 294

It is the most important type of fibro-osseous lesion for us, because it is the most common and we see it a lot.

• Occurs in tooth-bearing areas of the jaws
• Most common fibro-osseous lesion encountered in clinical practice
  
  • Three types:
    
    • Focal
    • Periapical
    • Florid
• For periapical- or florid cemento-osseous dysplasia, diagnosis can be made from the distinctive clinical & XRAY findings – do NOT need biopsy
  
  • In fact, biopsy of florid COD may lead to necrosis due to the hypovascularity
• FCOD (focal cemento-osseous dysplasia) may require surgical investigation because the features are less specific
• Encourage good oral hygiene to those with periapical or florid COD so they keep their teeth; extraction may lead to necrosis

Question 295

Answer 295

Cherubism / cental giant cell granuloma (but its bilateral)

Question 296

Vitamin B3 name and function?

Answer 296

Vitamin B3 (niacin): acts as a coenzyme for oxidation-reduction reactions. Pellagra= dermatitis, dementia, diarrhea.

Question 297

What two infections cause erythema multiforme minor?

Answer 297

Herpes simplex or Mycoplasma pneumoniae

Question 298

Which disease is associated with a “blow-out” or ballooning distention of the contour of the affected bone, with cortical expansion and thinning?

Answer 298

Aneurysmal bone cyst. Almost never found in jaw though, mostly long bones.

Question 299

Answer 299

Ewing’s Sarcoma

• Primary malignant tumor of bone (translocation of 11;22 chromosomes) -This line is the only thing we need to know for Ewing Sarcoma
• 3rd most common osseous neoplasm (after osteosarc & chondrosarc)
• 80% of patients are younger than 20
• M>F; C>AA
• Most affected: Long bones, pelvis, ribs
• Jaws are only involved 2% of the time
  
  • Pain, with associated swelling, is the most common symptom
  • If jaws are involved, paresthesia & loosening of the teeth are common findings; md>mx
• Via XRAY:
  
  • Irregular RL with ill-defined margins
  • Characteristic “onionskin” periosteal reaction (usually only seen in long bones, only rarely in jaws)

Question 300

How does the Orthokeratinized Odontogenic Cyst histopathology differ from the OKC?

Answer 300

Orthokeratinized Odontogenic Cyst has orthokeratinized lining and no basal palisading. Remember, OKC has parakeratin and a palisaded basal layer – Also, the granular cell layer is present in orthokeratinized, but not in parakeratinized, so it is found here, but not in odontogenic keratocyst.

Question 301

Answer 301

Idiopathic Osteosclerosis

• Focal area of increased radiodensity that is of unknown cause and cannot be attributed to anything else
  
  • Do not confuse with condensing osteitis, which is associated with an infection – the clue for condensing osteitis is that the tooth will have deep caries or a deep filling/root canal, because it is associated with an infection. If virgin tooth with no caries, then not condensing osteitis.
  • Do not confuse with focal cemento-osseous dysplasia, which will have a radiolucent rim
  • Do not confuse with cementoblastoma, which will be fused with the tooth
• Affects approximately 5% of Americans
• Most cases arise in teenage years and remain static
• Invariably asymptomatic
• No cortical expansion
• 90% in the mandible
• Via XRAY:
  
  • Well-defined, round or elliptical, radiopaque
  • Usually associated with root apex, 20% aren’t
  • Vary from 3mm to 2cm in greatest diameter
  • A radiolucent rim does NOT surround the lesion
• Diagnosis can be made based on history, clinical features, and radiographic findings
• Biopsy is considered only if there are symptoms, continued growth, or cortical expansion
• If lesion is discovered during adolescence, periodic XRAYs are prudent until the area stabilizes; after that, no treatment is necessary

Question 302

Answer 302

Paget’s Disease of Bone

• Relatively common disease characterized by abnormal resorption and deposition of bone
• Results in distortion and weakening of affected bones
• Unknown cause
• Affects older adults, M>F, C>AA
• 1 in 125 people over the age of 45
• Affects more than one bone (polyostotic)
• Bone pain is a common complaint
  
  • Pagetic bone forms near joints & promotes osteoarthritic changes, with associated joint pain and limited mobility
• Most commonly affected bones:
  
  • Vertebrae
  • Pelvis
  • Skull
  • Femur
• Involvement of weight-bearing bones leads to a bowing deformity, resulting in a simian (monkeylike) stance
• Paget’s disease affecting the skull leads to a progressive increase in the circumference of the head
• Jaw involvement occurs in 20%
  
  • Maxillary disease is much more common than mandibular disease
  • Results in an enlargement of the middle 1/3 of the face; severe cases results in leontiasis ossea, or lionlike facial deformity – this is for boards, not for his test.
  • Alveolar ridges are symmetrical and grossly enlarged
    
    • Patients classically complain dentures no longer fit (or, if present, increased space between teeth)
• Via XRAY:
• Patchy sclerotic areas are said to have a cotton wool or cotton roll appearance
• Teeth often have hypercementosis
• Patients have high elevations in serum alkaline phosphatase levels with NORMAL calcium and phosphorus levels – Know this
• The disease is slowly and chronically progressive, but rarely causes death
• Treatment is not given in asymptomatic patients with limited involvement
  
  • NSAIDs are given for pain; pharmacologic antiresorptive agents are given for symptomatic patients or those with alkaline phosphatase levels 50% above normal
    
    • Bisphosphonates are often used
• Development of a malignant bone tumor, osteosarcoma (biggest worry), is a recognized complication of Paget’s disease
  
  • Up to 10%
  • Most develop in the pelvis or long bones of lower extremities
  • Very aggressive and associated with poor prognosis

Question 303

Answer 303

Cherubism

Question 304

What type accounts for 80% of all Fibrous Dysplasia cases?

Answer 304

Monostotic

Question 305

The giant cell fibroma is generally unilateral. True or False?

Answer 305

False. It is typically bilateral.

Question 306

What are autoantibodies directed against with Pemphigus vulgaris?

Answer 306

Against desmosomes, which bond epithelial cells to each other, so you get a split within the epithelium. Cells of the epithelium “fall apart” – termed acantholysis. The loose cells are rounded and termed Tzanck cells.

Question 307

What is a positive Nikolsky sign associated with?

Answer 307

Pemphigus Vulgaris, and it is when bulla can be induced on normal-appearing skin if firm lateral pressure is exerted.

Question 308

Answer 308

Focal Osteoporotic Marrow Defect

• Area of hematopoietic marrow that produces a radiolucency
• May be confused with an intraosseous neoplasm
  
  • It is NOT pathology, but the XRAY features may look like it
• Typically asymptomatic & incidental finding on radiograph
• Radiolucent lesion that varies in size
• Typically has ill-defined borders with fine central trabeculations
• 75% in adult women, typically in posterior mandible
• No jaw expansion
• Incisional biopsy is necessary for diagnosis
  
  • Once dx is established, no treatment is necessary
  • No association between focal osteoporotic marrow defect and any hematologic disorder

Question 309

Answer 309

histo know the def. saw tooth rete pegs (lichen planus)

Question 310

Answer 310

Traumatic Bone Cyst

• Also known as simple bone cyst
• Benign, empty or fluid filled cavity within bone
• **“Cyst” is a misnomer because the lesion does not have an epithelial lining** - know this
• Trauma-hemorrhage theory is the most widely accepted etiology:
  
  • Trauma to the bone which is insufficient to cause a fracture results in intraosseous hematoma
  • If the hematoma does not undergo organization & repair, it may liquefy and result in a defect
• Majority of simple bone cysts are found in long bones
• In the jaws, they are found in pts aged 10-20
  
  • Almost exclusively found in mandible
  • Asymptomatic; 20% have a swelling
• XRAY:
  
  • Well-delineated
  • Radiolucent
  • Margins can be ill- or well-defined
  • **When several teeth are involved, the defect shows domelike projections that scallop between the roots; this feature is highly suggestive** - know this
• Teeth are vital
• Radiographic features are suggestive but not diagnostic
• Surgical exploration is necessary
  
  • Little or no tissue will be obtained, so the diagnosis is made with the clinical & radiographic features together with surgical findings
  • During surgery, the wall of the cavity should be curetted to rule out thin-walled lesions
• Surgical exploration is curative; there will rapid obliteration of the defect by new bone formation
• Periodic XRAYs should be taken until complete resolution has occurred; the prognosis is excellent

Question 311

Answer 311

Acanthosis Nigricans

(No clinical image, know this is seen in conjuction with GI cancer)

• An acquired dermatologic problem
• Characterized by the development of a velvety, brownish alteration of the skin
• It can be seen in conjunction with gastrointestinal cancer: - similar to internal malignancy associated with Lesser Trelat syndrome (associated with multiple cancers, not just GI)
  
  • Termed malignant acanthosis
  • The cutaneous lesion is benign
  • Leads to discovery of malignancy in 20% of cases – so not malignant in and of itself
• Most cases are benign acanthosis nigricans
  
  • Seen in 5% of adults and is caused by a variety of conditions
• Benign acanthosis nigricans may be caused by:
  
  • Obesity (termed pseudoacanthosis nigricans)
  • Inheritance
  • Various endocrinopathies (like diabetes mellitus)
  • Various syndromes (like Crouzon)
  • Various drugs (like oral contraceptives)
• Both benign and malignant acanthosis nigricans affect the flexural areas of the skin
  
  • Appear as finely papillary, hyperkeratotic, brown patches which are asymptomatic
  • The texture is either velvety or leathery
• Oral lesions of acanthosis nigricans may occur in up to 50%
  
  • Especially affect those with the malignant form
  • Lesions appear as diffuse, finely papillary areas of mucosal alteration that most often involve the tongue or lips
    
    • Especially the upper lip
  • The brownish pigmentation associated with the cutaneous lesions is usually not seen in the oral lesions
• The condition itself is harmless
• Patient should be evaluated for presence of disease or malignancy
• For those with malignant acanthosis nigricans:
  
  • Prognosis is poor
  • The lesions resolve with cancer treatment

Question 312

What are the characteristics of metastases to the oral soft tissues?

Answer 312

• Mechanism by which tumors can spread to the oral cavity is poorly understood
• Primary malignancies from adjacent tissues may spread via lymphatics
• Mets from the lower parts of the bodies are likely blood-borne and therefore would be expected to be found in the lungs
  
  • One possible explanation of H&N metastases in the absence of lung mets is Batson’s plexus
    
    • This is a valveless vertebral venous plexus that might allow retrograde spread of tumor cells & bypass the lungs
• Gingiva is the most common site for the soft tissues (50% of all cases), then tongue (25%) – 2/3 of metastatic disease goes to soft tissue, 1/3 goes to bone.
• Lesion appears as a nodular mass that resembles a hyperplastic growth, such as a pyogenic granuloma
• Most cases represent carcinomas rather than sarcomas
• Prognosis is poor

Question 313

Where do 50% of lipomas occur and what helps diagnose a lipoma?

Answer 313

On the buccal mucosa, and lipomas float in Formalin.

Question 314

What develops later in life? Ameloblastic carcinomas, conventional ameloblastomas, or malignant ameloblastomas?

Answer 314

Ameoblastic carcinomas

Question 315

What are the six common soft tissue sarcomas?

Answer 315

• Rare in the oral and maxillofacial region
• Account for <1% of cancers in the head and neck
• Include:
  
  • Fibrosarcomas (head and neck)
  • Malignant fibrous histiocytoma (now termed undifferentiated pleomorphic sarcoma)
  • Liposarcoma (thigh, retroperitoneum)
  • Leiomyosarcoma (uterus, GI)
  • Angiosarcoma (scalp and forehead)
  • Malignant peripheral nerve sheath tumor (MPNST)

Question 316

What percent of metastatic disease go to soft tissues vs bone? And most common soft tissue site?

Answer 316

2/3 soft tissue, 1/3 bone, and gingiva (50%)

Question 317

What is another name for Toxic Epidermal Necrolysis?

Answer 317

Lyell’s disease. This is the most severe form of erythemia multiforme.

Question 318

Answer 318

Cherubism

• Developmental jaw condition; AD inheritance
• The name cherubism was used because the facial appearance is similar to that of plump-cheeked little angels (cherubs)
  
  • Cherublike faces arises from bilateral involvement of the posterior mandible that produces chubby cheeks
  • There is an “eyes upturned to heaven” appearance due to a wide rim of exposed sclera noted below the iris
    
    • The exposed sclera is due to:
      
      • Involvement of the infraorbital rim & orbital floor = eyeballs tilted upward
      • Stretching of the upper facial skin = pulls lower lid downward
• Disease occurs between the ages of 2-5
• Clinical alterations progress until puberty, then stabilize & slowly regress
• Mandibular lesions are painless, bilateral, posterior, and expansile
• Maxillary involvement occurs posteriorly as well
  
  • In severe cases, entire mx & md are involved
• Causes marked widening and distortion of the alveolar ridges
• May lead to failure of tooth eruption amongst other things
• XRAY features:
  
  • Multilocular radiolucencies involving multiple quadrants of the mouth
  • Expansile
  • Radiolucent
    
    • The appearance is virtually diagnostic due to the bilateral symmetrical location in a young person – know this
• Microscopic findings are identical to those found in central giant cell granulomas
• Prognosis is unpredictable
  
  • Usually, the lesions show varying degrees of remission & involution after puberty
  • By age 30, most patient’s facial features are normal
  • However, some patients are left with facial deformities
• Early surgical intervention with curettage has lead to both good results or rapid regrowth with worsening deformity; therefore, optimal therapy hasn’t been determined
• Radiation therapy is contraindicated due to risk of postirradiation sarcoma

Question 319

Which lesion produces a “cupping” resorption of the underlying alveolar bone?

Answer 319

Peripheral Giant Cell Granuloma

Question 320

What is the most recently “named/discovered” odontogenic cyst?

Answer 320

Glandular Odontogenic Cyst

Question 321

What are autoantibodies directed against with Mucous Membrane Pemphigoid?

Answer 321

Against the basement membrane, leading to a subepithelail split. Direct immunofluorescence (DIF) also shows C3 and IgG along the basement membrane.

Question 322

At what age are eruption cysts most common?

Answer 322

In children younger than 10

Question 323

What is the other name for a Calcifying Odontogenic Cyst?

Answer 323

Gorlin Cyst

Question 324

If metastatic disease is excluded, what percentage of all malignancies that involve bone does Multiple Myeloma account for?

Answer 324

50%

Question 325

What are the characteristics of Acromegaly?

Answer 325

• Excessive production of growth hormone after the closure of the epiphyseal plates
• Increased growth of mandible (prognathism) with diastema formation, macroglossia

Question 326

Which pathology has the “target-like” appearance on the XRAY?

Answer 326

Osteoid Osteomas

Question 327

Answer 327

Inflammatory Papillary Hyperplasia (IPH)

• Reactive tissue growth that develops under a denture
• Condition is related to:
  
  • Ill-fitting denture
  • Poor denture hygiene
  • Wearing denture 24h a day
• 20% of patients who continuously wear their denture have IPH
• Typically occurs on the hard palate
• Asymptomatic, erythematous tissue with a pebbly or papillary surface
• Most patients also have a Candidal infection
• For very early lesions, removal of the denture may allow healing
• Condition may show improvement after antifungal therapy
• For more advanced lesions, treatment is surgical removal

Question 328

Answer 328

Erythema Migrans

• AKA Geographic tongue (but if on locations other than tongue, called erythema migrans)
• AKA Benign migratory glossitis
• Common condition primarily affecting the tongue
• Affects up to 3% of the population
• F:M 2:1
• May be related to psoriasis
• Characteristically, the lesions appear on the anterior 2/3 of the dorsal tongue
  
  • Concentrated on the tip & lateral borders
• May appear in other places but will have the serpentine border which helps with identification
• The lesions begin as multiple, small white patches which develop central erythematous atrophic zones and enlarge into well-demarcated zones of erythema
  
  • These areas are surrounded at least partially by a slightly elevated, yellow-white, serpentine or scalloped border
• The lesions will heal in one area and then develop in a different area
• There is an association with fissured tongue
• Most patients are asymptomatic but may experience burning when eating spicy foods
• No treatment necessary for asymptomatic erythema migrans
• No biopsy required, It is a clinical diagnosis
  
  • Patients should be assured they have a benign condition

If patients are symptomatic, topical corticosteroids are appropriate

Question 329

Answer 329

Central Giant Cell Granuloma

• Considered a nonneoplastic lesion
• Some demonstrate aggressive behavior
• Most cases occur before age 30; F>M; Md>Mx
• **More common in the anterior jaw; frequently cross the midline** - know this (the most common two things that cross the midline are this one and glandular odontogenic cyst)
• Most are asymptomatic & discovered during routine XRAY or due to painless bone expansion
  
  • A minority of cases are aggressive & associated with pain, paresthesia, & perforation of the cortical plate
• XRAY:
  
  • Not diagnostic
  • Radiolucent lesions which may be multilocular or unilocular
  • Typically well-delineated (different from corticated)
  • Noncorticated margins (usually slow growing ones have corticated rims, but this picture above is more aggressive and not slow so it corticated)
• The histopathology is identical to a brown tumor of hyperparathyroidism (so patients MUST be evaluated for this condition) and lesions of cherubism.
  
  • Histology is a cellular, vascular stroma with prominent multinucleated giant cells (Without the history of the radiograph, this image on the right looks like other things, like a peripheral giant cell granuloma)
• Treatment is curettage with a recurrence of 20% - if small enough
  
  • Likelihood of recurrence is greater in young patients
  • Most recurrent lesions respond to further curettage
• New treatments are used with aggressive tumors, such as injections of corticosteroids – if large
• Long term prognosis is good; there is no risk for metastasis

Question 330

Answer 330

Ameloblastoma (reverse polarity, apical vacuolization and palisading)

Question 331

What are the four main things considered as differentials for a bump on the gums?

Answer 331

1. Pyogenic Granuloma (a lot of capillaries on histo)
2. Peripheral Giant Cell Granuloma (giant cells on histo)
3. Peripheral Ossifying Fibroma
4. IFH (Inflammatory Fibrous Hyperplasia)

Just remember 3 P’s and IFH. These are very hard to differentiate, need a biopsy.

Question 332

With which nerve is the unilateral distribution located along with Sturge-Weber Angiomatosis?

Answer 332

Trigeminal Nerve (CN V)

Question 333

Answer 333

Thrombocytopenia

• Hematologic disorder characterized by a markedly decreased number of circulating blood platelets….the result may be abnormal bleeding (blood can’t clot, blood leaks out of vessels, etc)
• Decrease in platelets may be due to:
  
  • Reduced production
  • Increased destruction
  • Sequestration in the spleen
• Reduced platelet count:
  
  • Can be a result of various causes, such as infiltration of the bone marrow by malignant cells or the toxic effects of cancer chemo
• Increased destruction:
  
  • May be caused by an immunologic reaction
  • Often precipitated by drugs; heparin is the most common
  • Can be due to an autoimmune reaction
  • May be found as a component of a systemic disease, such as systemic lupus erythematous or HIV
  • Can be caused by abnormal blood clot formation, such as in patients with thrombotic thrombocytopenic purpura (TTP)
• Sequestration in the spleen:
  
  • Normally, 1/3 of the platelet population is sequestered in the spleen
  • Conditions causing splenomegaly = larger numbers of platelets to be taken out of circulation
• Normal platelet count is 200,000 – 400,000/mm3 – He won’t ask us about platelet counts
• Clinical evidence of thrombocytopenia is not seen until platelet count is below 100,000/mm3
• Condition is often initially detected due to the oral lesions
• In thrombocytopenia patients, the small capillaries leak blood which results in petechiae.
  
  • Petechiae = pinpoint hemorrhagic lesions
  • If a larger quantity of blood is extravasated, an ecchymosis will result rather than petechiae
  • Even larger amounts of extravasated blood results in a hematoma
    
    • (Hemat = blood; oma = tumor)
• Other oral lesions that can occur:
  
  • Spontaneous gingival hemorrhage
  • Bleeding from minor sites of trauma
• Platelet counts less than 10,000/mm3 is considered severe thrombocytopenia and can result in massive bleeding & death
• Special types of thrombocytopenia include TTP and idiopathic (immune) thrombocytopenic purpura (ITP)
  
  • ITP occurs in childhood, classically after a viral infection
    
    • 90% of cases resolve in 6 months
  • TTP is a more serious disorder of coagulation and is probably due to endothelial damage
  • Refer to physician for treatment; 70% survival

Question 334

What is the name of the lesion/granuloma that frequently occurs in pregnant women, most commonly in the 1st trimester?

Answer 334

Pyogenic granuloma, a.k.a. pregnancy tumor

Question 335

Which virus causes Kaposi’s Sarcoma and what are the four clinical presentations?

Answer 335

Caused by HHV-8

• Four clinical presentations:
  
  • Classic
  • Endemic (African)
  • Iatrogenic immunosuppression associated
  • AIDS related
• Classic type:
  
  • 80% in older men
  • Italian, Jewish, or Slavic descent
  • Multiple, asymptomatic blue-purple macules on the lower extremities which grow into nodules
• Endemic (African) has 4 subtypes
  
  • Benign nodular (similar to classic)
  • Aggressive
  • Florid
  • Lymphadenopathic (children)
• Iatrogenic:
  
  • Most common in organ transplant recipients
  • 0.5% of renal transplant patients

Question 336

How small are Osteoid Osteomas in comparison with Osteoblastomas?

Answer 336

Osteoid Osteomas are smaller than 2 cm usually, osteoblastomas are way bigger.

Question 337

How does histo differ for pemphigus vulgaris and mucous membrane pemphigoid?

Answer 337

The blue desmosomes are on the basement membrane not associated with the epithelial (which has detached from the underlying layers), they are attached to the underlying layerswith pemphigus vulgaris. With mucous membrane the blue desmosomes are attached to the epithelium still and the epithelium has detached.

Question 338

Answer 338

1. Dentigeour
   
   1. Dentigerous cyst
   2. OKC
   3. Ameloblastoma

Question 339

Why are the sclera, lingual frenum, and soft palate more affected by jaundice?

Answer 339

Elastin fibers have an affinity for bilirubin, so the following areas are more prominently affected:

Question 340

With what disease is a marfanoid build associated with?

Answer 340

MEN, type 2B

Question 341

On what part of the tongue does erythema migrans typically form?

Answer 341

On the anteiror 2/3 of dorsal tongue

Question 342

What is lichen planus called when it is confined to the gingiva?

Answer 342

If confined to the gingiva, it is termed desquamative gingivitis (differentials to desquamative gingivitis are pemphigus, pemphigoid, lichen planus, and hypersensitivity like Big Red)

Question 343

What is the characteristic feature of Granular Cell Tumors that are also exhibited in Blastomycosis?

Answer 343

Pseudoepitheliomatous Hyperplasia (PEH)

Question 344

Answer 344

Paget’s Disease of Bone

Question 345

What are the characteristics of Gigantism?

Answer 345

• Increased production of growth hormone, usually related to a functioning pituitary adenoma.
  
  • Takes place before the closure of the epiphyseal plates
  • Oral finding: generalized macrodontia

Question 346

What are the main four characteristics of Gardner Syndrome?

Answer 346

1. Skeletal abnormalities (usually Osteomas)
2. Dental Abnormalities (supernumerary teeth)
3. Epidermoid Cysts
4. Colonic Polyps/Adenocarcinoma

Question 347

What is the best known type of ectodermal dysplasia?

Answer 347

Hypohydrotic Ectodermal Dysplasia

Question 348

In what direction do odontogenic keratocysts tend to grow?

Answer 348

Anterior to posterior direction

Question 349

Answer 349

White Sponge Nevus

• Genodermatosis (a.k.a. genokertosis)= genetically determined skin disorder
• Autosomal dominant
• Defect in keratins (keratins 4, 13)
• Lesions appear at birth or early childhood
  
  • Symmetrical, thickened, white, corrugated, diffuse plaques on the bilateral buccal mucosa
  • Recognizable so not leukoplakia
  • May also occur in other parts of the oral cavity
  • Asymptomatic
  • Benign condition with no treatment

Question 350

What are the characteristics of Osteoblastomas and Osteoid Osteomas?

Answer 350

• Closely related, benign bone tumors
• Arise from osteoblasts
• Histopathologic features are identical
• **Osteoid osteoma produces prostaglandins; the pain is relieved by aspirin**
• **Distinction usually depends on the size of the lesion; osteoid osteomas are SMALLER than 2 cm; osteoblastomas are larger**
• Typically both lesions are treated by local excision or curettage
• Prognosis is good
• Sometimes lesions will regress after incomplete excision
• Lesions typically don’t recur unless they are aggressive osteoblastomas
• 50% recur

Question 351

What is the intrabony counterpart of the gingival cyst of the adult?

Answer 351

Lateral Periodontal cyst

Question 352

Answer 352

Mucous Membrane Pemphigoid

• May also be termed cicatricial pemphigoid; cicatrix means scar
  
  • Twice as common as pemphigus
• Avg age is 55; F:M 2:1
• Oral lesions begin as vesicles or bullae which eventually rupture and leave ulcerated mucosa
  
  • Intraoral blisters may be seen clinically (unlike with pemphigus); an intraoral blood blister is virtually pathognomonic
  • Unlike other lesions in this disease, oral lesions usually don’t scar
• Most significant complication is the ocular involvement (up to 25%)
  
  • Earliest change can be detected via slit-lamp examination by an ophthalmologist
  • As disease progresses, conjunctiva become inflamed and eroded; attempts at healing lead to scarring
  • Adhesions, called symblepharons, result
    
    • Scarring can turn the eyelids inward (entropion), which causes the eyelashes to rub against the cornea
    • Scarring can close opening of lacrimal glands
    • After all this happens, the cornea produces keratin as a protective mechanism
      
      • Keratin is opaque; this leads to blindness
    • Blindness can also occur by the upper and lower eyelids scarring together
• Histopathologic features:
  
  • Autoantibodies are directed against the basement membrane, leading to a subepithelial split (interepithelial)
  • Direct immunofluorescence (DIF) shows C3 and IgG along the basement membrane
• Treatment:
  
  • First part of treatment is to refer to an ophthalmologist, regardless of if the patient has symptoms
  • Topical corticosteroids are used first; if they are unsuccessful, systemic agents may be used

Question 353

Answer 353

Lateral Periodontal Cyst

• Developmental odontogenic cyst which typically occurs along the lateral root surface
• Arises from rests of dental lamina
• Intrabony counterpart of the gingival cyst of the adult (so the gingival cyst of the adult is the soft tissue counterpart)
• Found in pts ages 40-60
• Striking predilection to occur in the mandibular premolar-canine-lateral incisor area – know this for sure
• Teeth are vital
• Via XRAY, it appears as a well-circumscribed RL
  
  • Occasionally, the lesion may appear polycystic
    
    • These are termed botryoid odontogenic cysts – Just know this definition, it is a Lateral Periodontal Cyst that is multilocular.
      
      • Botroid means “grapelike”
      • Usually appear multilocular via XRAY
      • Represents a variant of the LPC
• Conservative enucleation is treatment
• Recurrence and/or malignant transformation is exceedingly rare

Question 354

What is the average age for Focal Cemento-Osseous Dysplasia?

Answer 354

40, middle-aged.

Question 355

What is the most common location of a fibroma?

Answer 355

The buccal mucosa along the bite line

Question 356

What are the XRAY characteristics of Osteopetrosis Infantile, and is Infantile or Adult more serious?

Answer 356

• Widespread increase in skeletal density (increased radiopaque)
• Roots of teeth are difficult to visualize because of the density of the surrounding bone
• Infantile is way more serious.

Question 357

What are the characteristics of Amyloidosis?

Answer 357

• Group of conditions characterized by the deposition of an extracellular, proteinaceous substance termed amyloid
• Several classifications exist:
  
  • Organ-limited
  • Systemic
  • Primary
    
    • Myeloma associated
  • Secondary
    
    • Hemodialysis associated
  • Heredofamilial
• Organ-limited:
  
  • Can occur in a variety of organs, but is rarely seen in the oral cavity
  • An example is an amyloid nodule, which is a solitary, asymptomatic, submucosal deposit
  • No association with any systemic condition
• Systemic – primary & myeloma-associated:
  
  • Affect older adults; M>F
  • 20% are due to multiple myeloma
  • Initial signs are nonspecific, but eventually carpal tunnel syndrome, mucocutaneous lesions, hepatomegaly, and macroglossia
  • Skin lesions appear as smooth-surfaced, firm, waxy papules and plaques
  • Most commonly affected areas are the eyelid, neck, and lips
• Systemic – secondary:
  
  • Develops as a result of a chronic inflammatory process, such as TB, sarcoidosis, or osteomyelitis
  • Spares the heart but affects the lever, kidney, spleen
  • Not common after advent of modern antibiotic therapy
• Systemic – hemodialysis-associated:
  
  • Protein isn’t removed by dialysis, so it accumulates in plasma
  • Eventually deposits in the bones & joints
• Systemic – Heredofamilial:
  
  • Uncommon but significant form of disease
  • AD inherited
  • Patients have polyneuropathies primarily
  • Other conditions, such as cardiomyopathy, cardiac arrhythmias, congestive heart failure, and renal failure develop as the condition progresses
• In most instances, no effective therapy is available
• Most patients with systemic forms succumb to cardiac failure, arrhythmia, or renal disease within months to a few years after the diagnosis

Question 358

Answer 358

Juvenile (Active) Ossifying Fibroma

• Distinguished from ossifying fibroma on basis of:
  
  • Age of patient
  • Most common sites of involvement
  • Clinical behavior
• Two different neoplasms have been reported under the term:
  
  • Trabecular
  • Psammomatoid
    
    • Psammatoid:trabecular 4:1
• Rapidly-growing
• Well-circumscribed
• Lesions are typically RL with central RO
• Both psammomatoid & trabecular:
  
  • M>F
  • Mx>Md
    
    • *Both of these characteristics are opposite of ossifying fibroma - know this
• Most tumors have a slowly progressive growth, but some exhibit rapid enlargement
• As a general rule, the younger the patient is, the more aggressive the tumor
  
  • Smaller lesions can be treated with local excision or curettage
  • Wide resection is required for rapidly enlarging or large lesions
• Recurrence has been reported in the range of 30-60%
• No malignant transformation

Question 359

What are the three types of Ameloblastoma?

Answer 359

1. Conventional solid or multicystic (85%)
2. Unicystic (15%)
3. Peripheral (1%)

Question 360

Where does a buccal bifurcation cyst most commonly develop?

Answer 360

Buccal aspect of the mandibular first permanent molar

Question 361

Answer 361

Calcifying Odontogenic Cyst

• AKA Gorlin Cyst
• The WHO reclassified this entity to be calcifying cystic odontogenic tumor, but most clinicians refer to this entity as a Gorlin cyst
• 65% are found in the incisor-canine areas; mx=md
• Average age of pt is 35
• 20% are associated with odontomas
• XRAY features:
  
  • Unilocular, well-defined radiolucency
  • Radiopaque structures are seen within the RL in 50%
• 1/3 are associated with an unerupted tooth (usually canine)
• Histopathologic features:
  
  • Has ghost cells, which can calcify
• Rare recurrences have been reported after enucleation
• Prognosis is good

Question 362

What is the most common way to get inflammatory papillary hyperplasia?

Answer 362

Ill-fitting denture, poor denture hygiene, wearing denture all day long. Epulis Fissuratum is similar but it is inflammatory fibrous hyplerplasia.

Question 363

Paget’s Disease of Bone usually only affects more than one bone. True or False?

Answer 363

True.

Question 364

Answer 364

Neuroma

• Can be either traumatic neuroma or palisaded encapsulated neuroma (PEN) – Just kind of know what this stands for
• Traumatic neuroma:
  
  • Not a true neoplasm; it is a proliferation of neural tissue after nerve injury
  • Most common in mental foramen area
  • 1/3 are painful
  • Surgical removal is typically curative

Question 365

What is the most common location of melanotic neuroectodermal tumors of infancy?

Answer 365

Anterior maxilla (61%)

Question 366

Polyps will result in cancer with Gardner Syndrome. True or False?

Answer 366

True. They will transform into adenocarcinomas.

Question 367

For which pathology are “loose bodies” seen on XRAY? Sometimes called joint mice.

Answer 367

Synovial Chondromatosis

Question 368

What are the oral manifestations of Tuberous Sclerosis?

Answer 368

Developmental enamel pitting on the facial aspect of the anterior permanent teeth, along with multiple fibrous papules.

Question 369

Which disease are the scalloped domelike projections between the roots associated with?

Answer 369

Traumatic Bone Cyst

Question 370

Where is the most common location in the mouth for a calcifying odontogenic cyst?

Answer 370

The Incisor-canine areas of both maxillary and mandibular.

Question 371

Answer 371

Orthokeratinized Odontogenic Cyst

• An odontogenic cysts with orthokeratinized lining and NO basal palisading
• Remember, OKC has parakeratin and a palisaded basal layer – Also, the granular cell layer is present in orthokeratinized, but not in parakeratinized, so it is found here, but not in odontogenic keratocyst.

Question 372

What is the most common clinically significant odontogenic tumor, because of its recurrence rate?

Answer 372

Ameloblastoma

Question 373

Answer 373

Non-Hodgkins Lymphoma

• AKA Lymphoma
• Most common lymphoma in mouth
• Diverse and complex group of malignancies
• Usually arise within lymph nodes and grow as solid masses (70%)
• 85% are of B-lymphocyte origin
• Occurs primarily in adults
• With nodal presentation, the patient is usually aware of a nontender mass that has been slowly enlarging for months
  
  • As the malignancy progresses, the nodes become more numerous and are fixed
    
    • Nodes become “fixed” because tumor cells violate the capsule and infiltrate the adjacent tissues
• Lymphomas may appear orally in either the soft tissues or within the jaws
• Intraoral soft tissue lesions typically appear as nontender, diffuse swellings affecting:
  
  • Posterior hard palate
  • Gingiva
  • Buccal vestibule
• Swellings are characteristically boggy
• Lesion may be erythematous or purple
• Patients who wear a denture may complain that their denture has become too tight
• Lymphoma of bone causes vague pain or discomfort which can be mistaken for a toothache
• The patient may have paresthesia, particularly with a mandibular lesion
  
  • Numb-chin syndrome
• XRAY changes:
  
  • Ill-defined or ragged radiolucency
• The most common type of lymphoma of the oral cavity is diffuse large B cell (DLBCL)

Question 374

Answer 374

Cementoblastoma

• Odontogenic neoplasm of cementoblasts
• 75% arise in the mandible, almost always in the molar/premolar region - know this
• Typically only affect permanent teeth
• 75% occur before age 30 - don’t need to know
• Pain and swelling are present in 2/3
• Via XRAY:
  
  • RO mass that is fused to one or more tooth roots – Can look very similar to cemento-osseus dysplasia, but this fact highlighted is a hint that this is the better answer.
  • Outline of the root or roots is usually obscured
  • Surrounded by a thin RL rim
• Treatment is surgical extraction of the tooth with the calcified mass – it will keep growing because it is a neoplasm, so we take it out.

Question 375

What is the average age for Periapical Cemento-Osseous Dysplasia?

Answer 375

40, middle-aged women.

Question 376

Answer 376

Ossifying fibroma (downward bowing) no picture. 2 types for juvenille trabecular and psammomotoid

Question 377

Answer 377

Odontogenic Keratocyst

• Histopathology: - Know these characteristics
• Thin, friable wall
• Epithelial lining is a uniform 6-8 layers thick
• Basal cell (dark blue) layer shows palisading (standing up like soldiers) & is hyperchromatic
• Epithelium is surfaced by wavy or “corrugated” parakeratin (has nuclei)
• May have small satellite cysts away from primary lesion – “daughter cysts” – another reason why recurrence rate is higher.

Question 378

Answer 378

Odontogenic Myxoma

(he won’t ask us to identify on radiograph because looks very similar)

• Myxomas only occur in the jaws
• Found in pts ages 25-30
• Md>Mx; M=F
• Small lesions are asymptomatic; large lesions are associated with a painless expansion of bone
• Via XRAY,
  
  • Unilocular or multilocular RL – may displace or cause resorption of teeth
  • RL defect may contain thin, wispy trabeculae of residual bone, which are often arranged at right angles to one another
• Small myxomas are treated by curettage with careful periodic re-evaluation every 5 years
• More extensive resection is required for larger lesions because myxomas tend to infiltrate the surrounding bone
• Recurrence is 25%, but prognosis is good

Question 379

Answer 379

Osteoma

• Benign tumors of mature bone
• Look very similar to tori
• Restricted to the craniofacial skeleton
• Arise on the surface of bone or within medullary bone
• Found in young adults as asymptomatic, solitary lesions
• Paranasal sinus lesions are more common than gnathic lesions
• May cause pain, swelling, sinusitis, or nasal discharge
• Via XRAY:
  
  • Circumscribed masses
  • Impossible to differentiate from small foci of sclerotic bone on initial XRAY
• Osteomas will exhibit continued growth
  
  • Usually RO, but can be RL with central RO, depending if they are compact or cancellous bone
    
    • Compact is RO; cancellous is mixed
• Large or symptomatic osteomas are treated by conservative excision
• Small, asymptomatic lesions probably do not need to be treated but should be observed and removed if lesions become large (interfere with function or esthetics) or symptomatic
• Completely benign with extremely rare recurrence
• Know Osteoma’s association with Gardner Syndrome below, we will not have to identify this radiographically because it is too similar to others

Question 380

Answer 380

Osteoma (cortical bone) assoc w/ gardners(polyps, supernumary teeth, osteomoas

Question 381

Answer 381

Aneurysmal Bone Cyst

• Intraosseous accumulation of blood-filled spaces surrounded by connective tissue
• **NOT a true cyst; no epithelial lining** - know this
• Etiology is unclear
• Typically seen in long bones; jaw lesions are uncommon
• Most jaw lesions are found in pts around age 20 in the posterior mandible; M=F
• Most common clinical manifestation is a swelling that has developed rapidly
• Pain is often reported
• XRAY:
  
  • Radiolucent lesion with marked cortical expansion and thinning
  • Usually unilocular; can be multilocular
  • Borders are variable; can be well-defined or diffuse
  • Frequently described is a “blow-out” or ballooning distention of the contour of the affected bone
• During surgery, venous blood wells up; there may be bleeding
• The appearance is like a “blood-soaked sponge”
• Treated by curettage or enucleation
  
  • Surgical defect typically heals within 1 year without the need of bone grafting
  • Recurrence ranges from 10-60% depending on the study and is usually due to inadequate removal of the first lesion
• Irradiation is contraindicated
• Prognosis is good

Question 382

Answer 382

Congenital Epulis

• Occurs almost exclusively on the alveolar ridges of newborns
• This is just a granular cell tumor
• Appears as a mucosal-colored, smooth-surfaced, polypoid mass that typically less than 2 cm
• 3x more common in the maxillary ridge
• Most frequently where the developing lateral incisor-canine area
• 90% occur in females
• No PEH; S-100 negative (different from granular cell tumor)

Surgical excision is curative, even with incomplete removal

Question 383

What is the other name for a Calcifying Epithelial Odontogenic Tumor?

Answer 383

Pindborg Tumor

Question 384

What are the three characteristics of Plummer-Vinson syndrome?

Answer 384

1. Iron-deficiency anemia
2. Glossitis
3. Dysphagia

Question 385

Vitamin B1 name and function?

Answer 385

Vitamin B1 (thiamin): maintain proper functioning of neurons. Beriberi.

Question 386

Is Burkitt’s lymphoma more common in children or adults?

Answer 386

Average age is 7; M>F – more common in children

Question 387

What is the complication that happens to 5% of people with NF1?

Answer 387

Up to 5% have Malignant Peripheral Nerve Sheath Tumors (MPNST), with an associated 5 year survival of 15%.

Question 388

What type of defect causes the white sponge nevus?

Answer 388

Keratins 4, 13

Question 389

What is another name for Multiple Endocrine Neoplasia, Type 2B?

Answer 389

Men 3

Question 390

What are the differentials for lesions that are mostly radiolucent with some calficiations?

Answer 390

Adenoomatoid odontogenic tumor, calcifying odontogenic cyst, calcifying epithelial odontogenic tumor, ameloblastic fibro-odontoma. If around impacted canine, it would be AOT. If it is a 10 year old child (you can tell from teeth), posterior mandible, impacted tooth, mixed radiolucency and radiopaque, then AFO is the answer.

Question 391

What type of Cafe au Lait is associated with NF1?

Answer 391

Coast of California

Question 392

What is the name for the primary malignant tumor of bone? And what chromosomal translocations are associated with it?

Answer 392

Ewing’s Sarcoma. Translocation of 11:22 chromosomes.

Question 393

What is the most common type of peripheral nerve neoplasm?

Answer 393

Neurofibroma

Question 394

Answer 394

central giant cell granuloma,, ameloblastoma, OKC ( glandular odontogenic?) (know differ)

Question 395

What happens to the tongue of half of the patients with NF1?

Answer 395

Enlargement of the fungiform papilla in 50%

Question 396

Answer 396

Ameloblastoma

“Honeycombed” appearance

• The typical radiographic feature is a multilocular radiolucency
  
  • The lesion is described in one of two ways:
    
    • “Soap bubble” – when the RL loculations are large
    • “Honeycombed” – when the loculations are small
    • **Buccal and lingual cortical expansion is frequently present**

Question 397

With Pemphigus Vulgaris what is Direct Immunofluorescence (DIF) positive for and what does Indirect immunofluorescence correlate with?

Answer 397

• Direct immunofluorescence (DIF) is positive for C3 and IgG within the epithelium
• Indirect immunofluorescence (IIF) correlates with disease activity

Question 398

Answer 398

Focal Cemento-Osseous Dysplasia

• Exhibits a single sight of involvement
• 90% occur in females
• Average age is 40 – middle-aged – this is the main age fact that we need to know for the test, no other ones
  
  • On your boards, this is more common in Caucasians. However, for your professional life, realize that is due to a population bias of the survey; it is actually more common in African Americans.
• Most common place is posterior mandible
• Asymptomatic
• Lesions are smaller than 1.5 cm
• Via XRAY:
  
  • Vary from completely radiolucent to densely radiopaque
  • Lesions will have a thin radiolucent rim
    
    • Differentiates from idiopathic osteosclerosis & condensing osteitis
  • Most commonly, there is a mixed RL-RO pattern
  • Lesion is usually well defined

Question 399

Answer 399

Granular Cell Tumor

• Benign soft tissue neoplasm that shows a predilection for the oral cavity
• Most common site is the tongue (up to 50%), with the dorsal surface being most common site – dorsal is mostly benign things
• Typically occurs around age 40; rare in kids
• 2:1 F:M
• Asymptomatic, sessile nodule that is less than 2 cm
• May be yellow or mucosal-colored
• African American patients may experience multiple tumors
• Significantly, these tumors exhibit significant pseudoepitheliomatous hyperplasia (PEH) – also with blastomycosis
  
  • If you submit a small dorsal tongue lesion for microscopic review and get back a reply of “squamous cell carcinoma,” kindly ask for a second opinion
• S-100 positive – this is a type of stain
  
  • Though the congenital epulis (discussed next) is also composed of granular cells, it is NOT associated with PEH and is S-100 negative
• Conservative excision is typically curative

Granular is increased lysosomes

Question 400

What are the characteristics of Fibro-Osseous Lesions?

Answer 400

• Diverse group of processes characterized by replacement of normal bone by fibrous tissue containing a mineralized product – there should be no fibrous connective tissue in bone, and only should happen with scarring or granulomas, or in this case, in response to a lesion.
• Microscopic lesions are very similar amongst different types of fibro-osseous lesions
• Therefore, clinical and radiographic finding are necessary to establish diagnosis
• Examples of BFOLs (benign fibro-osseous lesions):
  
  • Fibrous dysplasia
  • Cemento-osseous dysplasia
    
    • Focal
    • Periapical
    • Florid
  • Ossifying fibroma

Question 401

What is the most significant complication of Mucous Membrane Pemphigoid?

Answer 401

Ocular Involvement. You can get symblepharons, and entropion.

Question 402

What is the most common type of inherited bone disease?

Answer 402

Osteogenesis Imperfecta

Question 403

Besides supernumerary teeth, what other dental/oral anomalies are associated with Cleidocranial Dysplasia?

Answer 403

Cleft palate and delay or failure of eruption of permanent teeth.

Question 404

What is Crowe’s sign?

Answer 404

It is freckling in the axillary region and is one of the diagnostic criteria of NF1.

Question 405

Answer 405

erthyema multiforme

Question 406

Answer 406

Epidermolysis Bullosa

• Group of inherited blistering mucocutaneous disorders
  
  • Four types:
    
    • Simplex
    • Junctional
    • Dystrophic
    • Hemidesmosomal
• Simplex:
  
  • Blistering of hands and feet
  • Mucosal involvement is uncommon
  • No scarring
  • Good prognosis
• Junctional:
  
  • Severe blistering at birth
  • Granulation tissue around the mouth
  • Oral lesions are common
  • Often fatal
• Dystrophic:
  
  • Can be dominant or recessive
    
    • Dominant forms are usually not life threatening
      
      • Blisters on areas exposed to chronic trauma (knuckles, knees)
      • Scarring occurs
      • Mild oral manifestations
    • Recessive are severely debilitating
      
      • Blisters for with minor trauma (like eating)
      • Secondary infections occur because of the large surface area involved
      • If pt survives into teenage years, hands will be scarred into “mittens”
• Treatment varies with type; none is needed for mild cases
• Sterile drainage of large blisters and topical ABX are used for more severe cases
• Patients with severe disease usually succumb before adulthood

Question 407

What is the most common tumor of infancy?

Answer 407

Hemiangioma

Question 408

What are the two diseases associated with sharply demarcated Punched Out radiolucencies?

Answer 408

Multiple Myeloma and Langerhans Cell Histiocytosis.

Question 409

What is the main characteristic feature of the neurofibromatosis type II (NF2)?

Answer 409

Bilateral schwannoma of auditory-vestibular nerve.

Question 410

Which pathology looks very similar to tori?

Answer 410

Osteomas

Question 411

What are the characteristics of Iron Deficiency Anemia?

Answer 411

• Most common cause of anemia in the U.S and the World
• Develops from excessive blood loss, increased demand for RBC’s, decreased uptake of iron, decreased absorption of iron.
• 20% women of childbearing years (menorrhagia) from chronic blood loss.
• Oral manifestations: angular cheilitis, atrophic glossitis (bald tongue, due to trauma, etc, whereas pernicious anemia has a large beefy bald red tongue)
• Laboratory finding: hypochromic microcytic RBC’s

Question 412

Where is the most common location for oral lesions with schwannomas?

Answer 412

The tongue

Question 413

What are the two characteristics associated with pain from Osteoid Osteomas?

Answer 413

1. The pain is nocturnal
2. The pain is relieved by aspirin

Question 414

What is the characteristic finding in half of systemic lupuc erythematosis patients?

Answer 414

Malar rash (butterfly rash). Sunlight makes it worse. May lead to renal failure.

Question 415

Do 80% of jaw metastases occur in the maxilla or mandible?

Answer 415

Mandible

Question 416

What is the name for a malignant bone tumor of cartilage?

Answer 416

Chondrosarcoma

Question 417

Which virus that is transmitted by blood can lead to leukemia?

Answer 417

HTLV-1 virus

Question 418

What tooth do dentigerous cysts most commonly involve?

Answer 418

Mandibular 3rd molars. Rarely involve unerupted deciduous teeth.

Question 419

How do you treat a dentigerous cyst?

Answer 419

Enucleation of the cyst together with the unerupted tooth.

Question 420

Answer 420

1. Adenomatoid odontogenic tumor
   
   1. AOT
   2. CEOT
   3. COC
   4. AFO

Question 421

What are the characteristics of Malignant Ameloblastoma & Ameloblastic Carcinoma?

Answer 421

Malignant Ameloblastoma & Ameloblastic Carcinoma

• Terminology is confusing:
  
  • Malignant ameloblastoma has histologic features of a conventional ameloblastoma, but shows metastatic deposits (lung most common site) – should just be called metastatic ameloblastoma.
  • Ameloblastic carcinoma has cytologic malignant features and follows a markedly aggressive course

Ameloblastic Carcinoma

• Develop later in life than conventional ameloblastomas and malignant ameloblastomas
• Aggressive lesions; XRAY findings show ill-defined margins and cortical destruction

Question 422

Answer 422

Lymphoid Hyperplasia

• Enlargement of lymphoid tissue
• Typically due to infection
• May affect the lymph nodes, Waldeyer’s ring, or aggregates of lymphoid tissue scattered throughout the oral cavity
• Aggregates of lymphoid tissue are most commonly seen:
  
  • Oropharynx
  • Soft palate
  • Lateral tongue
  • Floor of mouth
• With acute infections, the lymphoid hyperplasia (tonsillar) appears as enlarged, tender, soft, freely movable nodules
• Chronic infection (tonsillar) produce enlarged, nontender, firm, freely movable nodules
• Chronic hyperplastic lymph nodes may be difficult to distinguish clinically from lymphoma
• Tonsillar size is variable from patient to patient
• Lymphoid tissue is usually more prominent in younger patients; peaking around age 20, then decreasing in size
• If large tonsils are symmetrical and asymptomatic, it is likely they are normal for that particular patient
  
  • Tonsillar asymmetry is a potentially serious sign that should be further evaluated to rule out a metastatic tumor or lymphoma
• Intraoral lesions appear as discrete, nontender, submucosal swellings
  
  • May be normal in color or yellow
  • If lesions appear on the posterior lateral tongue, they are usually bilateral
• Biopsy is usually necessary to establish diagnosis

Once diagnosis is confirmed, no treatment is required

Question 423

What is the name of a unifocal, monoclonal, neoplastic proliferation of plasma cells? And what does it probably give rise to?

Answer 423

Plasmacytoma. Multiple Myeloma.

Question 424

Answer 424

Multiple Endocrine Neoplasia, Type 2B (MEN 2B)

• Sometimes termed MEN 3
• A system complex characterized by: Know the highlighted ones
  
  • Parathyroid tumors
  • Pituitary tumors
  • Pancreatic tumors
  • Adrenal gland tumors (pheochromocytoma)
  • Thyroid tumors (medullary carcinoma)
  • Mucosal neuromas with a predilection for the oral cavity
• Patients have a marfanoid build with thin, elongated limbs
• Patients have a narrow face with characteristically thick and protuberant lips
• Oral mucosal neuromas are typically the first sign of the condition (!!!)
• Present as soft, painless papules (more superficial located) or nodules (deeper in connective tissue, but both are still bumps)
• Bilateral neuromas of the commissural mucosa are highly characteristic – Know this
• 50% develop pheochromocytoma
• 90% develop medullary carcinoma of the thyroid gland
• Diagnosed between the ages of 18-25 and has a marked propensity for metastasis (this age group doesn’t go to the dentist that often, so it is scary)

Question 425

What is another name for a Traumatic Bone Cyst?

Answer 425

Simple Bone Cyst

Question 426

Answer 426

Jaundice

• Excess bilirubin in the bloodstream accumulates in the tissues, which results in a yellowish discoloration of the skin & mucosa
• Numerous causes; some are physiologic, many are pathologic
• Gilbert syndrome is seen in 5% of Americans:
  
  • Inherited
  • Innocuous
  • Impaired processing of bilirubin by the liver
• Other causes:
  
  • Sickle cell anemia
  • Liver infections or toxins
  • Cancer
• Elastin fibers have an affinity for bilirubin, so the following areas are more prominently affected:
  
  • Sclera
  • Lingual frenum
  • Soft palate
• Other signs and symptoms due to hyperbilirubinemia may occur with jaundice:
  
  • Fever, abdominal pain, anorexia, fatigue

Patients should be evaluated for underlying condition; prognosis depends on the etiology of the jaundice

Question 427

What are the other two names for erythemia migrans?

Answer 427

• AKA Geographic tongue (but if on locations other than tongue, called erythema migrans)
• AKA Benign migratory glossitis

Question 428

What is another name for pernicious anemia?

Answer 428

Megaloblastic anemia

Question 429

What is Systemic Sclerosis?

Answer 429

A disease in which dense collagen is deposited in the tissues of the body in extraordinary amounts

Question 430

Which mineral levels are high and which mineral levels are normal in Paget’s Disease of Bone?

Answer 430

Patients have HIGH elevations in serum alkaline phosphatase levels with NORMAL calcium and phosphorus levels. Teeth often have hypercementosis.

Question 431

What is the S-100 positive test for?

Answer 431

Granular Cell Tumor, it is a type of strain. Though the congenital epulis is also composed of granular cells, it is NOT associated with PEH and is S-100 negative

Question 432

Of what cell origin is the malignancy coming from in Multiple Myeloma?

Answer 432

Plasma Cell origin

Question 433

Answer 433

Fibrous Histiocytoma

• True neoplasm exhibiting fibroblastic and histiocytic differentiation
• Occurs most commonly on the skin
  
  • On skin it is called dermatofibroma
• In the oral cavity, the buccal mucosa is the most common site
• Cutaneous lesions occur in young patients; oral lesions occur in older patients
• Appears as a painless nodular mass

Treatment is surgical excision

Question 434

What are the characteristics of Lupus Erythematous?

Answer 434

• Classic example of an immunologically mediated condition
• Most common collagen vascular or connective tissue disease in the US
• There are several clinicopathologic forms:
  
  • Systemic lupus erythematous (SLE)
  • Chronic cutaneous lupus erythematous (CCLE)
  • Subacute cutaneous lupus erythematous (SCLE)
  • Intermediate features between SLE & CLE
  • Will not discuss this form more than that
• In addition to clinical & microscopic features, immunologic studies are helpful in making the diagnosis of LE:
  
  • DIF of clinically normal skin will show IgG, IgM, or C3 at the BMZ; this is termed positive lupus band test
    
    • Other conditions may have this too; not specific
  • 90% have anti-nuclear antibodies (ANAs) – not specific, but very sensitive
  • Anti-dsDNA (70%)
  • Anti-Sm (30% - VERY specific)
    
    • Sm is a protein complexed with small nuclear RNA

Question 435

What pathology involves for the majority of the time the periapical region of the anterior mandible?

Answer 435

Periapical Cemento-Osseous Dysplasia

Question 436

What is the treatment for cementoblastomas?

Answer 436

• Treatment is surgical extraction of the tooth with the calcified mass – it will keep growing because it is a neoplasm, so we take it out.

Question 437

What is another name for Nevoid Basal Cell Carcinoma syndrome?

Answer 437

Gorlin Syndrome

Question 438

Answer 438

Lichen Planus

• Common, chronic dermatologic disease that can affect the oral mucosa
• May be due to medications, amalgam, etc. – this is better known as “lichenoid mucositis”
• Patients are middle-aged adults; F>M
• Approximately 1% of the population is affected
• Skin lesions are the 4 Ps:
  
  • Purple
  • Pruritus - itchy
  • Polygonal
  • Papules – slightly raised
    
    • Skin lesions itch, but the patient doesn’t usually scratch because they will hurt
    • The skin papules will also have a thin, lacelike network of white lines termed Wickham’s striae
• There are two forms of oral lesions:
  
  • Reticular
  • Erosive
• Lichen planus – Reticular – In most cases it is bilateral and that is a clue it is not leukoplakia
  
  • Much more common than erosive form
  • Asymptomatic
  • Involves the posterior buccal mucosa bilaterally, seen as Wickham’s striae
  • If it occurs on the tongue, the lesions will be plaque-like
  • Lesions tend to wax and wane
• Lichen planus - Erosive
  
  • Patients are symptomatic
  • Lesions are atrophic, erythematous areas with a central ulceration
    
    • Periphery is usually bordered by fine, white, radiating striae
  • If confined to the gingiva, it is termed desquamative gingivitis (differentials to desquamative gingivitis are pemphigus, pemphigoid, lichen planus, and hypersensitivity like Big Red)
    
    • Becomes indistinguishable from pemphigus & pemphigoid, which may also be limited to the gingiva
• Drug-induced LP lesions are typically found on the lower lip as non-healing ulcers
• Histopathologic features:
  
  • Pointed, “saw-toothed” rete ridges
  • Destruction of the basal layer
  • Band-like infiltrate of lymphocytes subjacent to the epithelium
  • Degenerating keratinocytes within the epithelium (termed Civatte bodies)
  • Deposition of fibrinogen at the basement membrane via DIF
• No biopsy is needed if the lesions are:
  
  • Bilateral AND asymptomatic
    
    • This is consistent with a clinical diagnosis of reticular oral lichen planus; no treatment is necessary
• Biopsy is indicated if the lesions are symptomatic or asymmetric
  
  • Oral topical corticosteroids are used for treatment
• A few extra tidbits to help your patients:
  
  • 28% of women with ELP have vaginal lesions
  • Lesions can be induced with the following:
    
    • S – Stress
    • T – Trauma
    • A – Advil (any NSAIDs, acetaminophen is ok)

Y – Yeast

Question 439

What is the name for inflammatory fibrous hyperplasia in association with the flange of an ill-fitting denture?

Answer 439

Epulis Fissuratum

Question 440

What are ill-defined, “moth-eaten” borders associated with?

Answer 440

Metastatic tumors to the jaw