Key Items Quiz 3 Flashcards
Tuberous sclerosis
Characterized by: CNS manifestation, Mental retardation, Seizure disorders, Potato-like growths (“tubers”), Angiofibromas of the skin, Ungual (or periungual) fibromas, characteristic skin lesions-Shagreen patches and Ash-leaf spots (ovoid area of hypopigmentation)
What is this
Odontoma
Most common odontogenic tumor
developmental anomalies (hamartomas) rather than true neoplasms
2 types: compound or complex
average age=15
Relatively small and discovered via XRAY when films are taken to determine the reason for failure of tooth eruption
Compound is more common in anterior maxilla; complex is more common in the molar regions
Compound-collection of tooth like stuctures varying in size and shape, surrouned by RL zone
Complex-calcified mass with RL similar to teeth, surrounded by RL rim
What is this
Amelobalstoma
Most common clinically significant odontogenic tumor
3 different types: Conventional solid or multicystic – 85%, Unicystic – 15%, Peripheral – 1%
Equal prevalence in the 3rd-7th decade
85% occur in the mandible, usually the molar-ascending ramus area – board question
If untreated, can grow to grotesque proportions – even then, pain & paresthesia are uncommon
typical radiographic feature is a multilocular radiolucency- “soap bubble” if large, “honeycombed” if small
buccal and lingual expansion is frequently present
root resorption is common
Desmoplastic form-predilection for anterior maxilla, RL-RO
Palisading and reverse polarity
Take 1.5cm margins beyond radiographic location
Polyostotic fibrous dysplasia
Jaffe-Lichenstien: polyostotic fibrous dysplasia and cafe au lait (coast of maine)
McCune-Albright: polyostotic fibrous dysplasia, cafe au lait (coast of maine), and multiple endocrinopathies (sexual percosity), pituitary adenoma and/or hyperthyroidism
Sexual percosity=early onset puberty
Aneurysmal bone cyst
Intraosseous accumulation of blood-filled spaces surrounded by connective tissue
NOT a true cyst; no epithelial lining
swelling that has developed rapidly
Pain is often reported
X-Ray: RL, coritcal expansion and thinning, usually unilocular but could be multilocular
during surgery “blood soaked sponge”
Schwannoma
aka Neurilemoma
benign neural neoplasm of of Schwann cell origin
50% of cases in H&N
Bilateral schwannomas of auditory-vestibular nerve are a characteristic feature of the neurofibromatosis type II (NF2)
tongue is the most common location for oral lesions
Antoni-A, organized, verocay bodies
Antoni-B, disorganized
Multiple Endocrine Neoplasia Type 2B (MEN 2B)
Charcterized by tumors of endocrine origin: parathyroid, pituitary, pancrease, adrenal gland (pheochromocytosis, 50%), thryoid (medullary carcinoma, 90%), mucosal neuromas
Marfanoid build
Oral mucosal neuromas are typically the first sign of the condition
Bilateral neuromas of the commissural mucosa are highly characteristic
Marked propensity for metastasis
Diagnosed between 18-25
Peutz-Jeghers syndrome
Rare but well recognized
Characterized by: Freckle-like lesions of the hands, perioral skin, and oral mucosa, Intestinal polyposis, Predisposition for affected patients to develop cancer
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
Intestinal obstruction due to intussusception
about 18x more likely to have a malignancy in their lifetime
GI adenocarcinoma also develops (but not from the polys)
Anemia
a decrease in volume of RBCs or in the concentration of hemoglobin
General symptoms: Tiredness, headache, fainting/feeling light headed, and pallor
Oral symptoms include: bald tongue (tongue atrophy), burning tongue (glossopyrosis), pain in the tongue (glossodynia)
Rhabdomyoma
Benign tumor of skeletal muscle
Extracardiac rhabdomyomas are very rare but have a predilection for the H&N
Adult and fetal types
70% of adult type are in men, nodular mass occuring in the pharynx or the floor of the mouth
Lymphoid hyperplasia
Enlargement of lymphoid tissue
May effect lymph nodes or Waldeyers’ ring
Common sites of lymph tissue in the oral cavity: oropharynx, soft palate, lateral tongue and floor of the mouth
Nevoid basal cell carcinoma syndrome
(Gorlin syndrome)
Chromosome 9 (PTCH gene)
Characterized by: Multiple basal cell carcinomas, OKCs, Calcification of falx cerebri, Bifid ribs
BCCAs appear around puberty and on skin not exposed to sunlight
OKCs are the most common feature-frequently multiple
Prognosis depends on skin tumors
Langerhans cell histiocytosis
Punched out radiolucency
teeth floating in air
Erythema Multiforme
Blistering, ulcerative mucocutaneous condition of uncertain etiopathogenesis
In 50% of cases, the clinician can identify a preceding infection or exposure to a medication
Infection is usually herpes simplex or Mycoplasma pneumoniae; medications are usually antibiotics or analgesics
spectrum=EM (minor), EM (major-steven-johnson’s syndrome), toxic epidermal necrolysis (lyell’s disease)
Acute onset
Prodromal symptoms (fever, headache, cough, sore throat) occur 1 week before onset
A highly characteristic skin lesion that develops is a target lesion
Hemorrhagic crusting of the vermilion zone of the lips is common
Steven-Johnson’s syndrome-usually triggered bu drug- To make this diagnosis, there must be skin, oral mucosa, and either ocular or genital mucosa involvement
Ossfying fibroma
True neoplasm with significant growth potential
composed of fibrous tissue that contains a variable mixture of bone & cementum
painless swelling
X-Ray: well defined, unilocular, mixed RL-RO, root divergence or resorption can occur
Large ossifying fibromas of the mandible demonstrate a characteristic downward bowing of the inferior cortex of the mandible
do not undergo malignant transformation
recurrence-rare
F>M
Gigantism/Acromegaly
Increased production of growth hormone, usually related to a functioning pituitary adenoma
Gigantism occurs before closure of epiphyseal plates, oral finding=macrodontia
Acromegaly, after closure of epiphyseal plates, Increased growth of mandible (prognathism) with diastema formation, macroglossia
Amyloidosis
deposition of extracellular amyloid
Organ limited-Rarely seen in the oral cavity, Amyloid nudule
Systemic-Primary and myeloma associated
20% are due to multiple myeloma, macroglossia, commonly affect eyelid, neck and lips
Systemic-secondary
Chronic inflammatory response
Systemic-hemodialysis associated
Protein isn’t removed in dialysis so it accumulates in plasma, eventually deposites in bone and joints
Metastasis to the oral soft tissues
One possible explanation of H&N mets 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 soft tissue
Usually carcinomas not sarcomas
2/3s of METs go to soft tissue and 1/3 to bone
Vitamin deficiencies
Vitamin A-Retinol-may lead to blindness
Vitamin B1-Thiamin-proper functioning of neurons-beriberi
Vitamin B2-riboflavin-cellular redox reactions-angular cheilitis and glossitis
Vitamin B3-Niacin-Pellagra=dermatitis, dimentia, diarrhea
Vitamin B6-prydoxine
Vitamin C-ascorbic acid-synthesis of collagen-scurvy
Vitamin D-hormone-calcium absorption-rickets and osteomalacia
Vitamin E-alpha tocopherol-antioxidant
Vitamin K-clotting-2,7,9,10-Coagulopathy because of inadequate synthesis of prothrombin
Calcifying Odontogenic Cyst (COC)
Gorlin cyst
calcifying cystic odontogenic tumor
65% are found in the incisor-canine areas; mx=md
X-Ray: Unilocular, well-defined radiolucency, Radiopaque structures are seen within the RL in 50%
Plummer-Vinson syndrome
Characterized by: iron-deficiency anemia, glossitis, and dysphagia
associated with a high frequency of both oral and esophageal squamous cell carcinoma
considered a premalignant condition
angular cheilitis often present
Spoon shaped configuration of nails (koilonchia)
5 to 50% chance of developing an upper aerodigestive tract malignancy
Lateral peiodontal cyst
Intrabony counterpart of the gingival cyst of the adult
Striking predilection to occur in the mandibular premolar-canine-lateral incisor area
X-Ray: well-circumscribed RL, could be polycystic (called botryoid odontogenic cyst)
CREST syndrome
C-calcinosis cutis
R-raynaud’s phenomenon
E-esophagal dysfuntion
S-Sclerodactyly
T-Telangiectasia
Osteoid Osteoma
Pain is nocturnal and relieved by aspirin
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
Gingival cyst of the adult
Soft tissue counterpart of the lateral periodontal cyst
derived from the rests of the dental lamina (rests of series)
Striking predilection to occur in the mandibular canine & premolar area (75% of the time)
Thrombocytopenia
Decreased number of circulating platelets
resulting in abnormal bleeding
Causes: decreased production, increased destruction, and sequestration in the spleen
Could be caused by bone marrow malignancies or chemo
Part of SLE or HIV
Might be caused by thromboitc thrombocytopenic purpura (TTP)
Normal levels 200,000-400,000
Symptoms when levels are below 100,000
Petechiae=small pinpoint hemorrhage
Oral problems-spontaneous gingival hemorrhage and bleeding from minor site of trauma
Osteopetrosis
Marble bone disease
hardening of the bone
marked increase in bone density
defect in remodeling caused by a failure of normal osteoclast function
2 types: infantile and adult
infantile=malignant osteopetrosis, wide spread increase in skeletal density
Adult=benign osteopetrosis
You see osteonecrosis because the bone grows and blocks blood flow
Could be seen as part of osteogenesis imperfecta
Central Giant Cell Granuloma
Non-neoplastic lesion
More common in the anterior jaw and frequenly cross the midline
Multi or unilocular
well delineated
non corticated margin
Histopathology is identical to brown tumor of hyperparathyroidism (must be evlauated) and lesions of cherubism
20% recurrence
no risk for metastasis
Hemangioma
Most common tumor of infancy
2 types: capillary and cavernous
Capillary: may not blanch clinically, usually red
Cavernous: typically blanches, darker red to purple
Cemento-osseous dysplasia
Occurs in tooth bearing areas of the jaw
most common fibro-osseous lesion encountered in clinical practice
3 types: focal, periapical, and florid
For periapical- or florid cemento-osseous dysplasia, diagnosis can be made from the distinctive clinical & XRAY findings – do NOT need biopsy
Multiple myeloma
Malignancy of plasma cell origin
Multicentric origin within bone
Second to metastatic malignancies, this is the most common malignancy of the bone
Most common hematologic malignancy of African Americans
X-Ray features: multiple “punched out radiolucencies”
Renal issues can be a sign because of the high volume of proteins in the blood called bence jones protiens
Cherubism
Developmental jaw condition
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
Occurs between the ages of 2-5
Clinical alterations progress until puberty and then stabilize and slowly regress
Bilateral CGCGs, posterior and expansile
Widening and expansion of alveolar ridges
X-Ray: multilucular, expansile, radiolucent
Systemic sclerosis
A disease in which dense collagen is deposited in the tissues
First sign of disease is Raynaud’s phenomenon-Vasoconstrictive event triggered by emotional distress or exposure to cold
Common cause of death is pulmonary fibrosis causing pulmonary hypertension and heart failure
Microstomia occurs as a result of collagen deposition in the perioral tissues
Xerostomia is frequently encountered
Dental appliances are used to combat microstomia
What are 2 types of neuromas?
Traumatic and palisaded encapsulated neruoma
Lupus Erythematous
Immunologically mediated
Most common collagen vascular or connective tissue disease in the US
several types but know these 2-Systemic lupus erythematous, Chronic cutaneous lupus erythematous
SLE-Malar rash-50%
Kidney problems (most significant issue)
Cardiac problems (pericarditis)- Up to 50% have warty vegetations affecting heart valves termed Libman-Sacks endocarditis
Oral lesions are lichenoid lesions
Treatment with antimalarial drugs (hydroxychloroquine)
Most common cause of death is kidney failure
Idiopathic osteosclerosis
Increased radiodensity
Don’t confuse with focal cemento-osseous dysplasia-radioluncent rim
Don’t confuse with cementoblastoma-fused with the tooth
90% in mandible
Hodgkin’s lymphoma
Malignant lymphoproliferative disorder
Neoplastic cells are called Reed-Sternberg cells (1-3% of cell of the lesion)
Binucleated “owl eye”
Significant percentage linked to EBV
75% occur in the cervical and supraclavicular nodes
Eruption cyst
Soft tissue analogue of the dentigerous cyst
Occurs within the soft tissues overlying the alveolar bone
Usually seen in children younger than 10
blue or purple color
Iron deficieny anemia
Most common cause of anemia
develops from excessive blood loss
oral manifestations-angular cheilitis and atrophic glossitis
Microcytic
Gardner sydrome
Think of osteoma
Colonic polyps/adenocarcinomas
Skeletal abnormalities
Dental abnormalities
Epidermoid cyst
Bowel polyps develp during teenage years, WILL transform into adenocarcinomas (50% by 30, 100% later in life)
Skeletal abnormalities-osteomas are most common, precede bowel polyps
Dental abnormalities-supernumerary, impacted teeth, and odontoma
Leukemia
malignancies of hematopoietic stem cell derivation
malignant transformation of a hematopoietic stem call that invades the bone marrow and over flows into the periphral blood
Chonic/acute and myeloid/lymphoid
Acute can lead to death in a few months if not treated
CML-translocation of long arms of 9 & 22 (philadelphia choromosome)
Viruses-HTLV-1
ALL common in childhood
CLL most common in adults
If leukemic cells enter the oral cavity they can cause a “boggy” non tender swelling historically called a granulocytic sarcoma
Rhabdomyosarcoma
Malignancy of skeletal muslces
60% of soft tissue sarcomas in childhood
Painless, infiltrative mass that grows rapidly
Traumatic bone cyst
Simple bone cyst
Benign empty of fluid filled cavity in the bone
“Cyst” is a misnomer because the lesion does not have an epithelial lining
Trauma causes an intraosseous hematoma, doesn’t repair properly
Majority are found in long bones
10-20 years old
almost exclussively in mandible
When several teeth are involved, the defect shows domelike projections that scallop between the roots; this feature is highly suggestive
Teeth are vital
Juvenile (active) ossifying fibroma
M>F (opposite of ossifying fibroma)
Psammomatoid:trabecular 4:1
RL with central RO
MX>MD
recurrence 30-60%
no malignant transformation
Osteoblastoma
md>mx
posterior>anterior
between 2-4 cm
Pain, tenderness, and swelling are important and common presenting features (but not relieved by aspirin)
RL with varying degrees of central RO
Florid cemento-osseous dysplasia
Multiple focal involvement not limited to the anterior mandible
90% are female; 90% are African American
Marked tendency to be bilateral and symmetrical
X-Ray features are similar to other 2, RL rim
Paget’s disease of bone
Abnormal resorption and deposition of bone
results in distortion and weakening of affected bones
Polyostotic
CC: bone pain
Pagetic bone forms near joints, promotes osteoarthritic changes
Weight bearing bones get a bowing deformity
if skull is affected, it leads to an increase in the circumference of the head (hat no longer fits)
Maxillary more common than mandibular
Enlargement of middle 1/3 of face, resulting in leontiasis ossea (lion like face)
X-Ray: patchy sclerotic areas (cotton wool or cotton roll appearance, hypercementosis,
Have elevated serum alkaline phosphatase levels, with normal calcium and phosphorus levels
Can develop malignant bone tumor osteosarcoma
Cleidocranial dysplasia
Dental and clavicle abnormalities
Increased prevalence of cleft palate
prolonged retention of deciduous teeth
delayed or failure of eruption of permanent teeth
Numerous unerupted permanent and supernumerary teeth
Periapical cemento-osseous dysplasia
Periapical regions of anterior mandible
90% female, 70% African Americans
early lesions look like periapical cyst or granuloma, with time the turn mixed RL-RO, and then densly RO with RL rim
PDL will be intact, will not fuse to tooth
Focal cemento-osseous dysplasia
90% occur in females
average age 40
On boards more common in caucasians
Posterior mandible
X-Ray: RL to densly radiopaque, thin RL rim, mixed RL-RO, well defined
Cementoblastoma
Odontogenic neoplasm of cementoblasts
75% arise in the mandible, almost always in the molar/premolar region
X-Ray: RO mass that is fused to one or more tooth roots, outline of the root or roots is usually obscured, surrounded by a thin RL rim
Fibrous dysplasia
Tumor like condition
replacement of normal bone with fibrous CT intermixed with bone
Results from a postzygotic mutation
could be mono or polyostotic
Mono-ostotic fibrous dysplasia
80% of all cases
Maxilla > mandible
X-Ray: ground glass opacification (poorly calcified bone in disorganized fashion)
Not well demarcated
Dentigerous cyst
3mm or less is normal area surrounding an erupting tooth
Unerupted tooth
fluid between the reduced enamel epithelium and the tooth crown
Most common developmental cyst
Encloses the crown of the unerupted tooth and is attached at the CEJ
Most often involve mandibular 3rd molars
X-Ray: Unilocular radiolucency associated with crown of unerupted tooth, Well-defined and usually sclerotic border
Calcifying Epithelial Odontogenic Tumor (CEOT)
AKA Pindborg Tumor
MD>MX
posterior>anterior
Most common presenting sign is a painless, slow-growing swelling
X-Ray: uni (more common in maxilla) or multilocular, Margins are typically scalloped & well-defined, Frequently associated with an impacted tooth, driven-snow
Amyloid-like extracellular material; positive for Congo red, which exhibits an apple-green
Calcifications with concentric rings form in the amyloid-like areas (Liesegang rings)
Metastatic tumors to the jaw
Most common origin of gnathic mets: Breast, lung, thyroid, prostate, kidney
Older patients
80% of jaw mets occur in the mandible
If close to the IA it can produce numb-chin syndrome
Can be discovered in non-healing EXT sites
X-Ray: ill defined boarder “moth eaten”
Ectodermal dysplasia
Group of inherited conditions in which two or more ectodermal derived anatomic structures fail to develop: Skin, hair, nails, teeth, sweat glands
Best known is hypohidrotic ectodermal dysplasia
X-linked
Periocular wrinkling with hyperpigmentation
Hypoplastic or absent salivary glands
Teeth are markedly reduced in number and have abnormal crown shapes (conical)
Buccal bifurcation cyst
Characteristically develops on the buccal aspect of the mandibular first permanent molar
1/3 have bilateral involvement
X-Ray: Well-circumscribed, unilocular radiolucency involving the buccal bifurcation and root, An occlusal XRAY can demonstrate buccal location, The root apices are characteristically tipped toward the lingual mandibular cortex
Treated by enucleation
Focal osteoporotic marrow defecy
Area of hematopoetic marrow that produces a radiolucency
may be confused with an intraosseous neoplasm
NOT pathology
ill defined boarder with fine central trabeculations
posterior mandible
Pernicious anemia
Megaloblastic anemia
caused by poor absorption of cobalamin (vitamin B12 extrinsic factor)
lack intrinsic factor because of autoimmune destruction of the parietal cells of the stomach, which results in decreased absorption of cobalamin
atrophic glossitis
Mucous Membrane Pemphigoid
aka cicatricial pemphigoid
Twice as common as pemphigus
Average age=55
Intraoral blisters may be seen clinically (unlike with pemphigus); an intraoral blood blister is virtually pathognomonic
Most significant complication is the ocular involvement- Adhesions, called symblepharons, result, Scarring can turn the eyelids inward (entropion), which causes the eyelashes to rub against the cornea
Langerhan’s cell histiocytosis
dendritic mononuclear cells-epidermis, lymph nodes, mucosa and bone marrow
antigen presenting cells
more than 50% of patients are younger than 15 years old
Birbeck granules differentiate these from other APCs
Radiographically-“punched out radiolucencies”, teeth look like they are “floaiting in air”
Osteosarcoma
Mesenchymal malignancy
ability to produce osteoid or immature bone
Excluding hematopoietic neoplasms, it is the most common type of malignancy to originate within bone
intramedullary, juxtacortical, or extraskeletal
extragnathic, bimodal age distribution: 10-20 (more common) and after 50
Osteosarcomas of the jaws comprise 7% of all osteosarcomas, around age 33
X-Ray:May be RO, RO with RL border, mixed, or RL, peripheral border is ill-defined, 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, A triangular elevation of periosteum termed Codman’s triangle can occur, Widened PDL around multiple teeth
Pemphigus Vulgaris
4 diseases but vulgaris (most common) is the one that affects the mouth
oral lesions are the “first to show and last to go”
Autoantibodies are directed to desmosomes
without treatment, oral and cutaneous lesions are progressive
characteristic feature is a positive Nikolsky sign: Bulla can be induced on normal-appearing skin if firm lateral pressure is exerted
Tzank cells (similar to herpes)
Direct immunofluorescence show postive C3 and IgG
Hailey-Hailey disease-Familial pemphigus
Changes in tonsils during acute and chronic infections
Acute-enlarged, tender, soft and freely movable
Chronic-enlarged, firm, non-tender, freely movable
hard to distinguish from lymphomas
Lymphoid tissue is usually more prominent in younger patients; peaking around age 20, then decreasing in size
Tonsillar asymmetry is a potentially serious sign that should be further evaluated to rule out a metastatic tumor or lymphoma
What is the difference between osteoblastoma and osteoid osteoma?
Osteoid osteoma produces prostaglandins (cause pain); the pain is relieved by aspirin (Enough prostaglandins to cause pain but enough the aspirin works, once it gets bigger the aspirin doesn’t help)
Distinction usually depends on the size of the lesion; osteoid osteomas are SMALLER than 2 cm; osteoblastomas are larger
Adenomatoid Odontogenic Tumor (AOT)
Young patients 10-20
uncommon over age of 30
striking predilection for anterior jaws
MX>MD
F:M, 2:1
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
RL often extends apically past the CEJ; this can help differentiate from dentigerous cyst
fine, snowflake calcifications via XRAY
Odontogenic Keratocyst (OKC)
2 most important things: 30% recurrence rate, associated with a syndrome (Gorlin syndrome)
Arise from cell rests of the dental lamina
AKA keratocystic odontogenic tumor (KOT)
Posterior mandible
Tend to grow in an anterior-posterior direction
X-Ray: smaller lesions-unilocular RL with corticated margins, large lesions-multilocular
unerupted tooth is involved in 1/3 of cases
Thin friable wall, epithelial cells 6-8 layers thick, palisading, hyperchromatic, parakeratin
daughter cysts (high recurrence rate)
Leiomyoma
Benign tumor of smooth muscle
Most oral lesions are a vascular variant (angioleiomyoma)
2 special types of thrombocytopenia
ITP (immune thrombocytopenic purpura)-childhood following viral infection
TPP (thrombotic throbocytopenic purpura)-serious disorder of coagulation and is probably due to endothelial damage
Lichen Planus
Skin lesions are the 4 Ps: Purple, Pruritus, Polygonal, Papules (Wickham’s striae)
2 oral forms: reticular (more common) and erosive
Reticular-Wickham’s striae
Erosive-Lesions are atrophic, erythematous areas with a central ulceration
If confined to gingiva it is called desquamative gingivitis
Histo: “saw tooth rete ridges”, band like infiltrate of lymphocytes subjacent to epithelium
White sponge nevus
Defect in keratins (keratins 4, 13)
Lesions appear at birth or in early childhood
Symmetrical, thickened, white, corrugated, diffuse plaques on the bilateral buccal mucosa
Benign condition with no treatment
Osteoma
Benign tumor of mature bone
restricted to craniofacial skeleton
paranasal sinus lesions are more common than gnathic
X-ray: circumscribed masses
exhibit continued growth
if compact bone RO: if cancellous mixed RO-RL
Completely benign with extremely rare recurrence
