Exam 3 Flashcards

Question

Vitamin B2 name and function?

Answer 1

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

Answer 2

Hemodialysis

Answer 3

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\*\*

Answer 4

Nevoid Basal Cell Carcinoma syndrome (Gorlin Syndrome)

Answer 5

Aneurysmal bone cyst

Answer 6

Cementoblastoma

Answer 7

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).

Answer 8

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

Answer 9

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

Answer 10

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.**

Answer 11

Odontoma- compound

Answer 12

Skin lesions

Answer 13

Stevens-Johnson syndrome

Answer 14

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

Answer 15

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)

Answer 16

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

Answer 17

Renal disease

Answer 18

Between 10 and 20 years old. 1. Distal Femur 2. Proximal Tibia

Answer 19

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

Answer 20

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

Answer 21

* 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

Answer 22

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.

Answer 23

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

Answer 24

Traumatic bone cyst (simple bone cyst)

Answer 25

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

Answer 26

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

Answer 27

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

Answer 28

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

Answer 29

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”

Answer 30

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

Answer 31

The development of a malignant bone tumor, osteosarcoma.

Answer 32

Botryoid (grapelike) Odontogenic Cyst

Answer 33

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

Answer 34

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)

Answer 35

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.

Answer 36

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

Answer 37

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”**

Answer 38

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

Answer 39

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

Answer 40

Mandibular premolar-canine-lateral incisor area.

Answer 41

Ameloblastic Fibroma

Answer 42

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

Answer 43

* 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

Answer 44

Lateral periodontal cyst ( botryoid-grapelike)

Answer 45

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

Answer 46

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

Answer 47

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

Answer 48

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

Answer 49

Multiple Myeloma. Average age is 65.

Answer 50

Odontogenic Keratocyst

Answer 51

Calcifying Epithelial Odontogenic Tumor

Answer 52

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

Answer 53

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

Answer 54

Buccal bifurcation cyst

Answer 55

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

Answer 56

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

Answer 57

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.

Answer 58

Florid cement osseus dysplasia

Answer 59

Buccal mucosa

Answer 60

Retrocuspid papilla

Answer 61

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)

Answer 62

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

Answer 63

Osteosarcoma

Answer 64

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

Answer 65

Vitamin E (α-tocopherol): antioxidant.

Answer 66

Paget's Disease of Bone

Answer 67

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

Answer 68

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

Answer 69

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

Answer 70

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

Answer 71

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

Answer 72

Pericarditis (libman-sacks endocarditis)

Answer 73

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%

Answer 74

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

Answer 75

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

Answer 76

* 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.

Answer 77

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

Answer 78

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

Answer 79

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.

Answer 80

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

Answer 81

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

Answer 82

Oral and esophageal squamous cell carcinoma

Answer 83

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

Answer 84

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

Answer 85

Von Recklinghausen's disease of the skin

Answer 86

Melanotic Neuroectodermal Tumor of Infancy

Answer 87

Paranasal sinus lesions

Answer 88

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."

Answer 89

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 y**ellow 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

Answer 90

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

Answer 91

desmosomes, pemphigus vulgaris(histo picture)

Answer 92

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

Answer 93

Gingival Cyst of the Adult

Answer 94

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

Answer 95

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

Answer 96

Osteopetrosis

Answer 97

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

Answer 98

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

Answer 99

* 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.***

Answer 100

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**

Answer 101

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

Answer 102

Lichen Planus

Answer 103

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

Answer 104

Eruption cyst

Answer 105

Chromosome 9, PTCH gene

Answer 106

Osteosarcoma

Answer 107

Oral lesions. First to show, last to go.

Answer 108

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)

Answer 109

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

Answer 110

OKC (tooth displaced)

Answer 111

* 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

Answer 112

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%)

Answer 113

* 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

Answer 114

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.**

Answer 115

Cell rests of the dental lamina

Answer 116

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

Answer 117

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

Answer 118

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

Answer 119

* **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

Answer 120

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**

Answer 121

* 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

Answer 122

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

Answer 123

Gardner Syndrome

Answer 124

Osteoid osteoma (nocturnal pain)

Answer 125

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

Answer 126

Gorlin syndrome

Answer 127

In teenage years.

Answer 128

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

Answer 129

Painful tongue

Answer 130

Type 1 (NF1)

Answer 131

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

Answer 132

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.

Answer 133

Keratocystic Odontogenic Tumor (KOT)

Answer 134

Children, age 10

Answer 135

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

Answer 136

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

Answer 137

False, usually bilateral.

Answer 138

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.

Answer 139

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

Answer 140

It is a benign tumor of smooth muscle, and is most common in 1. Uterus 2. GI tractt 3. Skin

Answer 141

* **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

Answer 142

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

Answer 143

PGCG has usually a bluish hint.

Answer 144

Calcifying Epithelial Odontogenic Tumor

Answer 145

In the incisor-cuspid region

Answer 146

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

Answer 147

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

Answer 148

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

Answer 149

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

Answer 150

Gingival cyst of the adult

Answer 151

Mandibular Molars

Answer 152

Burning of tongue (associated with anemia)

Answer 153

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.

Answer 154

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

Answer 155

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

Answer 156

In the lumbar spine.

Answer 157

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

Answer 158

In the anterior jaw

Answer 159

Langerhans Cell Histiocytosis

Answer 160

* 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.

Answer 161

Developmental anomaly (hamartomas)

Answer 162

Mandibular canine/premolar area

Answer 163

Yes, they are vital

Answer 164

Ameloblastic Fibrosarcoma

Answer 165

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

Answer 166

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.

Answer 167

* 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

Answer 168

False. No association.

Answer 169

Paget's Disease of Bone

Answer 170

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

Answer 171

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

Answer 172

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

Answer 173

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

Answer 174

Burkitt's Lymphoma

Answer 175

In long bones. Almost exclusively in the mandible.

Answer 176

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

Answer 177

Cleidocranial Dysplasia and Gardner Syndrome

Answer 178

False. Neither of them are.

Answer 179

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

Answer 180

Marble Bone Disease

Answer 181

* 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

Answer 182

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

Answer 183

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

Answer 184

Between 3rd to 7th decade

Answer 185

Ameloblastic fibroma

Answer 186

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

Answer 187

Central Giant Cell Granuloma

Answer 188

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

Answer 189

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

Answer 190

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

Answer 191

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

Answer 192

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

Answer 193

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

Answer 194

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

Answer 195

Type I collagen

Answer 196

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

Answer 197

Idiopathic Osteosclerosis

Answer 198

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

Answer 199

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

Answer 200

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

Answer 201

Granular Cell Tumor

Answer 202

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

Answer 203

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

Answer 204

* 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

Answer 205

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

Answer 206

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

Answer 207

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

Answer 208

* 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)

Answer 209

On the gingiva or edentulous alveolar ridge.

Answer 210

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

Answer 211

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

Answer 212

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

Answer 213

More mild disease is seen in patients who: * Have a better histocompatibility match * Are young * Are female * Have received cord blood

Answer 214

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

Answer 215

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

Answer 216

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

Answer 217

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

Answer 218

Glandular Odontogenic Cyst and Odontogenic Keratocyst

Answer 219

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

Answer 220

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

Answer 221

Dermatofibroma

Answer 222

* 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

Answer 223

Cemento-Osseous Dysplasia

Answer 224

1. Eyelid 2. Neck 3. Lips

Answer 225

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**

Answer 226

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

Answer 227

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

Answer 228

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

Answer 229

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

Answer 230

Eruption Cyst

Answer 231

False. It is, and has significant growth potential.

Answer 232

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.

Answer 233

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

Answer 234

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

Answer 235

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

Answer 236

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

Answer 237

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

Answer 238

Tongue and buccal mucosa

Answer 239

Osteoblastoma or Osteoid Osteoma

Answer 240

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

Answer 241

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

Answer 242

OKC (know histo!) easy cheese

Answer 243

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**

Answer 244

* 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

Answer 245

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**

Answer 246

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

Answer 247

* 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

Answer 248

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

Answer 249

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)

Answer 250

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

Answer 251

75%, typically in posterior mandible

Answer 252

Dentigerous Cyst

Answer 253

Mandible, anterior jaw (crosses midline).

Answer 254

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

Answer 255

Sporadic condition resulting from a postzygotic mutation

Answer 256

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

Answer 257

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

Answer 258

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

Answer 259

Cherubism / cental giant cell granuloma (but its bilateral)

Answer 260

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

Answer 261

Herpes simplex or Mycoplasma pneumoniae

Answer 262

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

Answer 263

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)

Answer 264

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.

Answer 265

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

Answer 266

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

Answer 267

Monostotic

Answer 268

False. It is typically bilateral.

Answer 269

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.

Answer 270

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

Answer 271

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**

Answer 272

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

Answer 273

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

Answer 274

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

Answer 275

* 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

Answer 276

On the buccal mucosa, and lipomas float in Formalin.

Answer 277

Ameoblastic carcinomas

Answer 278

* 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)**

Answer 279

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

Answer 280

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

Answer 281

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

Answer 282

Peripheral Giant Cell Granuloma

Answer 283

Glandular Odontogenic Cyst

Answer 284

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

Answer 285

In children younger than 10

Answer 286

Gorlin Cyst

Answer 287

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

Answer 288

Osteoid Osteomas

Answer 289

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

Answer 290

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

Answer 291

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

Answer 292

Ameloblastoma (reverse polarity, apical vacuolization and palisading)

Answer 293

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.

Answer 294

Trigeminal Nerve (CN V)

Answer 295

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**

Answer 296

Pyogenic granuloma, a.k.a. pregnancy tumor

Answer 297

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

Answer 298

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

Answer 299

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.

Answer 300

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

Answer 301

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

Answer 302

MEN, type 2B

Answer 303

On the anteiror 2/3 of dorsal tongue

Answer 304

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

Answer 305

Pseudoepitheliomatous Hyperplasia (PEH)

Answer 306

Paget's Disease of Bone

Answer 307

* **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**

Answer 308

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

Answer 309

Hypohydrotic Ectodermal Dysplasia

Answer 310

Anterior to posterior direction

Answer 311

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

Answer 312

* 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

Answer 313

Lateral Periodontal cyst

Answer 314

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

Answer 315

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

Answer 316

40, middle-aged.

Answer 317

The buccal mucosa along the bite line

Answer 318

* 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.

Answer 319

* **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

Answer 320

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

Answer 321

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

Answer 322

Buccal aspect of the mandibular first permanent molar

Answer 323

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

Answer 324

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

Answer 325

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

Answer 326

Anterior maxilla (61%)

Answer 327

True. They will transform into adenocarcinomas.

Answer 328

Synovial Chondromatosis

Answer 329

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

Answer 330

Traumatic Bone Cyst

Answer 331

The Incisor-canine areas of both maxillary and mandibular.

Answer 332

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.

Answer 333

Ameloblastoma

Answer 334

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)**

Answer 335

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.**

Answer 336

40, middle-aged women.

Answer 337

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

Answer 338

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.

Answer 339

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

Answer 340

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**

Answer 341

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

Answer 342

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

Answer 343

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

Answer 344

Pindborg Tumor

Answer 345

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

Answer 346

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

Answer 347

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

Answer 348

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

Answer 349

Keratins 4, 13

Answer 350

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.

Answer 351

Coast of California

Answer 352

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

Answer 353

Neurofibroma

Answer 354

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

Answer 355

Enlargement of the fungiform papilla in 50%

Answer 356

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\*\*

Answer 357

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

Answer 358

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

Answer 359

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

Answer 360

* 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

Answer 361

Ocular Involvement. You can get symblepharons, and entropion.

Answer 362

Osteogenesis Imperfecta

Answer 363

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

Answer 364

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

Answer 365

erthyema multiforme

Answer 366

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

Answer 367

Hemiangioma

Answer 368

Multiple Myeloma and Langerhans Cell Histiocytosis.

Answer 369

Bilateral schwannoma of auditory-vestibular nerve.

Answer 370

* **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

Answer 371

The tongue

Answer 372

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

Answer 373

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

Answer 374

Chondrosarcoma

Answer 375

HTLV-1 virus

Answer 376

Mandibular 3rd molars. Rarely involve unerupted deciduous teeth.

Answer 377

Enucleation of the cyst together with the unerupted tooth.

Answer 378

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

Answer 379

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

Answer 380

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

Answer 381

Plasmacytoma. Multiple Myeloma.

Answer 382

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)

Answer 383

Simple Bone Cyst

Answer 384

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

Answer 385

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

Answer 386

Megaloblastic anemia

Answer 387

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

Answer 388

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

Answer 389

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

Answer 390

Plasma Cell origin

Answer 391

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

Answer 392

* 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

Answer 393

Periapical Cemento-Osseous Dysplasia

Answer 394

* 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.

Answer 395

Gorlin Syndrome

Answer 396

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

Answer 397

Epulis Fissuratum

Answer 398

Metastatic tumors to the jaw