Pathology Flashcards
What are epithelial origin benign tumors?
-Ameloblastoma (Conventional solic, multicystic, unicystic, extraosseous/peripheral)
-Squamous odontogenic tumor
-Calcifying epithelial odontogenic tumor
-Adenomatoid odontogenic tumor
What are mixed (epithelial and mesenchymal) origin tumors?
-Ameloblastic fibroma
-Primordial odontogenic tumor
-Odontoma (compound, complex, dentinogenic ghost cell tumor)
What are mesenchymal origin tumors?
-Odontogenic fibroma
-Odontogenic myxoma
-Cementoblastoma
-Cemento-ossifying fibroma
What are malignant odontogenic tumors?
-Ameloblastic carcinoma
-Primary intraosseous carcinoma, not otherwise specified
-Sclerosing odontogenic carcinoma
-Clear cell odontogenic carcinoma
-Odontogenic carcinosarcoma
-Odontogenic sarcoma
What are the types of odontogenic cysts?
-Developmental: Dentigerous, OKC, lateral perio/botryoid cyst, gingival cyst, glandular odontogenic cyst, calcifying odontogenic cyst, orthokeratinized odontogenic cyst
-Inflammatory: Radicular, collateral inflammatory cyst
Describe ameloblastoma
Three types:
-Conventional (solid, multi-cystic)
-Unicystic
-Extra-osseous (peripheral)
What age group do you expect to see ameloblastoma?
Occur at any age.
50% of all cases are between 20-40 years old
What demographic do you see ameloblastoma?
No sex predilection
20-40 year old
80% in mandible (75% molar/ramus region)
20% in maxilla (mostly posterior)
What are some pre-op features that can present with ameloblastoma?
Slow growing, expansile odontogenic epithelial neoplasm
See dental changes: Mobility, displacement, root resorption
What are radiographic features of ameloblastoma?
Multilocular radiolucency (can also be unilocular
20% associated with unerupted tooth
What is the histology of ameloblastoma?
-Cords, strands of islands of epithelium. Palisading reverse nuclear polarity subnuclear vacuoles) set in fibrous stroma
-Central portion resembles stellate reticulum
-Most have follicular or plexiform pattern (plexiform often don’t have classic features)
What is the treatment of ameloblastoma?
-Ameloblastoma in situ: Cyst enucleation (only one)
-Mural (limited to epithelial lining of cyst), intraluminal (arrising in epithelial lining and proliferating into lumen), microinvasive (invading to basement membrane), invasive
-Requires resection with 1-1.5 cm of bony margins and one uninvolved anatomic barrier
-Rarely extends more than 8 mm past radiographic margin.
What is the cure and recurrence rate of ameloblastoma?
98% cure from resection
Recurrence of 70-85% for enucleation and curettage due to incomplete removal
What is desmoplastic ameloblastoma?
-Older group, anterior jaw, maxilla>mandible
-Radiographs suggest fibro-osseous lesion
What is unicystic ameloblastoma?
-May be more aggressive
-5% of all ameloblastomas
-Occur in younger age range (10-24 years), can be from transformation of reduced enamel epithelium remnants (dentigerous cyst or OKC)
What is peripheral ameloblastoma?
-Arising from oral surface epithelium (gingival)
-Not aggressive, does not invade bone
-Low recurrence rate
What is sinonasal ameloblastoma?
-Males, 61-year olds, from pluripotential cells of sinonasal epithelium
-Presents with nasal obstruction and epistaxis
-Usuallh plexiform, no metastasis
What are the molecular features of ameloblastoma?
-Mutations of MAPK pathway in 90% of ameloblastomas.
-BRAF-gargeted therapy in aggressive/recurrent ameloblastomas
What are the clinical features of squamous odontogenic tumor?
-Young adults 20-40
-No sex predilection
-Mandible=maxilla, posterior mandible and anterior maxilla, 20% have multiple lesions
-Originates from rests of Malassez
-Expansile, swelling of alveolus, mobility/tenderness
What are the radiographic features of squamous odontogenic tumor?
-Well defined semicircular or triangular radiolucent defect around the roots of affected teeth, similar appearance t juvenile perio and eosinophilic granuloma
What is the histology of squamous odontogenic tumor?
-Bland islands of squamous epithelium in fibrous stroma
-No evidence of ameloblastic features
-Histo Ddx: Desmoplastic ameloblastoma and SCCa
-May have calcifications or eosinophilic masses (PAS+)
What is the treatment of squamous odontogenic tumor?
Complete surgical excision through curettage, recurrence has been reported
What are the clinical features of calcifying odontogenic tumor?
-Age 2nd to 10th decades, mean age 40
-No gender predilection
-Mandible 2x more likely than maxilla, 3x more likely posterior mandible to anterior mandible
-Expansile, asymptomatic swelling of jaw
What are the radiographic features of CEOT?
-Variable. uni vs multilocular radiolucency. Mixed ensity lesion, often with an unerupted tooth, eventual expansion of the cortical plates
What is the histology and IHC of CEOT?
-Irregular sheets of islands, amyloid (congo red), ring calcifications
-Can look like carcinoma
-IHS: +Cytokeratin, amyloid, sometimes S100
What is the treatment of CEOT?
-Complete surgical excision/thorough curettage/en bloc resection
>20% recurrence rate
What are the variants of CEOT?
-Peripheral CEOT (gingiva w/o bone usually anterior jaws)
-Clear cell and pseudoglandular architecture (older pt, 6% of cases)
-6% associated with AOT
What are the clinical features of adenomatoid odontogenic tumor (AOT)?
-Age 5-30 years, mean 18 years old
-Female 2x than male
-75% are female under 21
-80-85 % in anterior jaw, 2x maxilla
-Slow growing, innocuous
What are the radiographic features of AOT?
Well circumscribed radiolucency, 75% associated with unerupted tooth
Radiographic flecks may be present
What is the histology of AOT?
-Whirled spindled round odontogenic cells, PAS+, thick fibrous capsule, may co-exist with CEOT
What is the treatment of AOT?
Enucleation and curettage, no recurrence
What are the clinical features of ameloblastic fibroma?
-Mean age 15, 40% under 10
-No gender predilection
-2% of tumors
-75% posterior mandible
-Expansile, asymptomatic swelling of the jaw
-May be associated with delay in eruption
What are the radiographic features of ameloblastic fibroma?
-Well demarcated uni/multi-locular radiolucency, often expansile
-Often associated with unerupted tooth
What is the histology of ameloblastic fibroma?
Thin strands/cords of ameloblastic epithelium w/o stellate reticulum
Also contains odontogenic mesenchyme
+Vimentin and ghost cells
What is the treatment of ameloblastic fibroma?
Complete excision/en bloc resection (simple curettage is insufficient)
-15-20 % recurrence rate
What are variants of ameloblastic fibroma?
Ameloblastic fibrosarcoma (malignant)
-Either de novo or transformation of ameloblastic fibroma
What are the clinical features of primordial odontogenic tumor?
-Very rare (less than 30 cases)
-Usually young patients
-Around crown of an impacted tooth
What are the radiographic features of a primordial odontogenic tumor?
-Well circumscribed pericoronal radiolucency
-Often associated with an unerupted tooth or in place of a tooth
What is the histology of a primordial odontogenic tumor?
-Dental papilla like tissue cuboid-columnar epithelium
-No dental hard tissue
What is the treatment of primordial odontogenic tumor?
-Limited data, conservative but complete enucleation
What are the clinical features of an odontoma (includes ameloblastic fibro-odontoma and ameloblastic fibrodentinoma)
-Wide age range, mean is 15
-No gender predilection
-More common anterior maxilla, complex common in posterior jaws
-Rarely can be extragnathic (middle ear)
-Most common odontogenic tumor (may be a hamartoma)
-Asymptomatic typically seen with delayed eruption of teeth
What are the radiographic features of an odontoma?
-Classically a well circumscribed radiolucent mass with a narrow radiolucent rim (may be mixed density)
-Compound: Multiple tooth-like structures in a halo of dental follicle
-Complex: Amorphous mass of enamel, dentin and pulp with dental follicle
What is the histology of an odontoma?
-All components of odontogenesis present
-Either well formed tooth structure (compound) or haphazard arrangement of products (complex)
What is the treatment of an odontoma?
Conservative surgery of enucleation
Describe the clinical features of ameloblastic fibro-odontoma?
Variant of odontoma
-Age 10-12 years, 60% under 10
-No sex predilection
-60% mandible, 40% maxilla
-Expansile, asymptomatic
What is the radiographic/histology of a ameloblastic fibro-odontoma?
-Well circumscribed mixed density
-Strands of islands and cords, looks a little like odontoma
-Variant of ameloblastic fibrodentinoma contains dentin like material
What is the treatment for ameloblastic fibro-odontoma?
Conservative surgery with curettage
Describe the clinical features of dentinogenic ghost cell tumor
-20-70 years old
-May present as non-healing extraction site, jaw expansion, painful swelling or ulcerated mass
-Extraosseous are less aggressive
What are radiographic signs of dentinogenic ghost cell tumor?
-Irregular, destructive mixed lesion
-May have opacification of sinus
-Solid neoplastic version of a COC (Calcifying odontogenic cyst)
What is the histology of dentinogenic ghost cell tumor?
-Resembles ameloblastoma in association with COC, ghost cells and dystrophic mineralization
What is the treatment of dentinogenic ghost cell tumor?
-Wide surgical resection; may metastasize
What are the clinical features of odontogenic fibroma?
-Wide range, mean is 40
-Strong female predilection
-60% anterior to 1st molar
-40% mandible, mostly posterior
-Slow growing, locally expansile neoplasm
-Associated with CGCG
What are the radiographic features of odontogenic fibroma?
-Well demarcated radiolucency, usually at root of tooth
-Displacement, resorption may be present
What is the histology of odontogenic fibroma?
-Proliferation of bland fibrous tissue and scattered rests of odontogenic epithelium
-Differential: hyperplastic dental follicle, desmoplastic fibroma of bone, infantile myofibromatosis
What is the treatment of odontogenic fibroma?
-Conservative local excision or curettage is sufficient
What are the clinical features of odontogenic myxoma?
-Age 10-50, mean 20-30
-No gender predilection
-Posterior mandible is most common site (but also seen in maxilla)
-Expansile, slow growing neoplastm
-Loosening of teeth common
What are the radiographic signs of odontogenic myxoma?
-Multilocular, well demarcated, can be ill defined
-Can have honeycomb trabecular pattern
-Root displacement not resorption
What are the histologic properties of a myxoma?
-Dental papilla, delicate myxoid connective tissue, +vimentin, +S100
What is the treatment of odontogenic myxoma?
-Aggressive odontogenic tumor
-Recurrence around 25%
-Resection with 1-1.5 cm margins and anatomic barrier
What are the clinical features of cementoblastoma?
-Benign odontogenic neoplasm of cementoblasts that make cementum
-8-44 with mean age 21-30
-Slight female predilection
-Site permanent teeth, particularly mandibular molars
-Usually presents with pain and expansion
What are the radiographic features of cementoblastoma?
-Well defined radiopaque mass adhered to the root with a thin radiolucent halo, no PDL seen between lesion and root
What is the histology of cementoblastoma?
-Dense basophilic, cementum like tissue
What is the treatment of cementoblastoma?
-Surgical removal of tooth and lesion
-Need to completely remove lesion or can recur
What are the clinical features of ameloblastic carcinoma?
-Histologic/behavior of malignant ameloblastic neoplasm, no calcified material
-Mean age 30
-Mets to lung (75%) or lymph nodes/spine (15%)
What are the radiographic features of ameloblastic carcinoma?
-Aggressive ill-defined radiolucency
-May expand the cortical plates, erode them and resorb roots
What is the histology of ameloblastic carcinoma?
-Atypia and malignancy in neoplastic ameloblastic epithelium
-No dentin, enamel, or cementum
What is the treatment of ameloblastic carcinoma?
Staged and treated as carcinoma involving the jaws
What are the clinical features of a dentigerous cyst?
-Most common developmental odontogenic cyst
-Always associated with the crown of an impacted or unerupted tooth
-From accumulation of fluid between crown and reduced enamel epithelium
-Most common with 3rd molars and canines
-Usually well definied radiolucency
-Thin connective tissue wall lined with squamous epithelium
What is the treatment for a dentigerous cyst?
Surgical removal (may recur), removal of impacted tooth, may give rise to ameloblastoma, SCCa, mucoepidermoid carcinoma
What are the variants of a dentigerous cyst?
Eruption cyst, primordial cyst
What are the clinical features of an OKC?
-Any age, mostly 2-3rd decade
-1/2 are symptomatic
-More common in mandible posterior area
-High recurrence
What are the radiographic features of an OKC?
75% unilocular (25% multi-locular)
-May have tooth displacement or resorption
What is the histology of an OKC?
Corrugated parakeratinized stratified squamous epithelium, 6-8 cells thick, palisaded basal layer
What is the treatment for OKC?
Conservative to aggressive surgical removal
-Peripheral ostectomy depending on anatomy
-Recurrence high due to incomplete removal, satellite cysts
What is nevoid basal cell carcinoma syndrome?
-Gorlin syndrome
-AD inheritence, variable penetrance
-Numerous basal cell carcinoma of skin, multiple OKC, palmar/plantar pits, enlarged calvarium, frontal bossing, calcified falx cerebri
-9q22 gene
What is the treatment of lateral periodontal cyst/botryoid odontogenic cyst?
-Surgical excision
-Botryoid more likely to recur
What is gingival cyst of infants?
-Smooth white nodules along crest of maxillary/mandibular alveolar ridge
-Don’t confuse with epstein pearl (midline palatal fissure cyst) or BOhn nodule (blocked salivary duct)
-No treatment needed, rupture with eruption of teeth
What are the features of glandular odontogenic cyst (clinical, radiographic, histology)?
-Mean age 50, anterior mandible
-Radiograph: Uni/multilocular radiolucency
-Similar to LPC, botryoid odontogenic cyst, central muco-epi (multicystic lesion with swirled appearance), mucous cells
What is the treatment of glandular odontogenic cyst?
-Wide local exicion/resection due to possibility of recurrences
What are the clinical/raiographic/histological features of calcifying odontogenic cyst?
-Any age (bimodal 3rd decade, lesser peak 6-7th decade)
-male=female
-maxilla=mandible
-intraosseous jaw swelling/gingival tenderness
-periphearl 30%
-Radiographic: Uni/multi-locular radiolucency w/ or w/o radiopacities
-Histology: Cyst with flattened columnar basal cells, ghost cells, possible calcifications
What is the treatment for calcifying odontogenic cyst?
Surgical excision
-May have recurrence or malignant transformation
-If associated with aggressive odontogenic tumor or cyst, treated as a neoplasm
Describe orthokeratinized odontogenic cyst?
-Not the same as OKC
-Less likely to recur than OKC
-Male predilection
-Surgical removal
Describe a radicular/periapical cyst.
-Most common odontogenic cyst
-Non-vital tooth
-Radiolucency at apex of tooth
-Source of inflammation must be resolved. Monitor after RCT, may need curettage after extraction
Describe clinical features of a chondrosarcoma.
-Less than 5% occur in jaws
-3rd-6th decade
-Maxilla and nasal septum> mandible
-M=F
-Slow growing, loosening of teeth
-Moth eaten uni/multi-locular radiolucency
-root resorption, cortical perforation
-50-60% IDH1/2 mutation
What is the treatment of chondrosarcoma?
-Wide excision with clean margins if possible
-Histologic grade and clear margins most predictive factors
-20% 5 year survival
What are the features of mesenchymal chondrosarcoma?
-Biphasic neoplasm of small blue round malignant cells
-M=F 4th decade
-Jaws most common site
-Destructive radiolucent mixed lesion
-Small blue round mesenchymal cells, SOX9 IHC
What is the treatment of mesenchymal chondrosarcoma?
-Complete resection
-Relatively good prognosis if completely resected, late metastases are seen requiring long term follow-up
What are the features of osteosarcoma?
-20% of all sarcomas, 5% occur in jaw (1-2 decades later than long bones)
-Male>female
-Mean age 40
-Mandible>maxilla
-Swelling with pain, loosening of teeth, paresthesia
-Can arise from pre-existing condition (fibrous dysplasia, radiation therapy, paget’s disease)
-Radiolucent to radiopaque, widening of PDL, sunburst pattern, moth-eaten appearance
What other processes are associated with increased incidence of osteosarcoma?
-Li Fraumeni syndrome, retinoblastoma, Werner syndrome, Rothmond-Thompson syndrome, giant cell tumor, chronic osteomyelitis, OI
Describe histological findings with osteosarcoma.
-Must have atypical mesenchymal cells producing osteoid
-Degree of atypia can correspond to differentiation
-Malignant cells produce cartilage/chondroid and/or collagen and can be classified as chondroblastic, osteoblastic, fibroblastic
-Low, intermediate and high grade
Describe the treatment of osteosarcoma.
-Prognosis correlated to complete resection and margin status
-6-21% can metastasize
-Complete resection with 3 cm margins and uninvolved anatomic barrier
-Adjunctive chemo for high grade lesions
-50% 5 year survival, 80% if clear margins on first surgery
Describe Paget’s disease of bone.
-Chronic, slow growing disease.
-3 phases: Resorptive, vascular, sclerosing), male>female, older than 50, deep bone pain and warmth
-Complaint of ill fitting denture
-Increased Alk Phos, increased urinary calcium
-Cloud like radiopaque/radiolucent areas, obliteration of lamina dura and PDL
Describe fibrous dysplasia
-Skeletal anomaly where normal bone is replaced by cellular fibrous connective tissue (immature, poorly mineralized)
-Adolescents 10-20 years old
-No sex predilection
-No race predilection
-Maxilla>zygomatic/temporal>mandible
What are the types of fibrous dysplasia?
Monostotic
Craniofacial
Polyostotic
What is monostotic fibrous dysplasia?
70% of cases involving a single bone
-Jaws, ribs, femur and tibia most common sites
-Young adults
What is craniofacial fibrous dysplasia?
-Slightly younger age group
-Usually more than one bone but can be mandible only
-No endocrine dysfuntion
What is polyostotic fibrous dysplasia?
-30% of cases
-Younger patients 2/3 under 10
-Can involve 3/4 of skeleton
-3% of all fibrous dysplasia cases can present with an endocrinopathy (mostly McCune Albright Syndrome of Jaffe’s Syndrome)
What is McCune Albright and Jaffe’s syndrome?
McCune Albright: Polyostotic fibrous dysplasia with cafe au lait spots, endocrine abnormalities (precocious anemia)
Jaffe’s syndrome: Polyostotic fibrous dysplasia with skin pigmentation
What are radiographic/histologic features of fibrous dysplasia?
-Expansion of jaw, ground glass/orange peel radiopaque appearance
-Ill defined borders
-Cortical bone replaced by lesional tissue
-Displaced teeth
Histology: Fibrous proliferation with C shaped bone
What is the treatment of fibrous dysplasia?
-Conservative therapy
-Cosmetic recontouring
-Complete removal not practical or necessary
-50% regrowth (need long term follow-up)
-Radiation contraindicated due to sarcomatous transformation
-Malignant transformation has occurred in absence of radiation
Describe cemento-osseous dysplasia
-3 entities (florid, periapical and focal cemento-osseous dysplasia)
-Non-neoplastic (reactive), process
-Wide age range (mean 40)
-Female and African American predilection
-Teeth are vital, asymptomatic, may have expansion
What is the radiographic/histologic features of cemento-osseous dysplasia?
Periapical: At root apicies (shows radiolucent rim)
Focal: Focal, more irregularity, usually involve root apices
Florid: Dense sclerotic masses in multiple quadrants
Histology: Fibro-osseous material, Hemorrhage (Blood lakes)
What is the treatment of cemento-osseous dysplasia?
-Diagnosis based on clinical radiographic features
-Follow-up is treatment
-A case of malignant transformation has been reported
-Avoid biopsy because can result in secondary osteomyelitis
What is familial giantiform cementoma?
-Rare autosomal dominant hereditary condition
-Rapid growth, massive jaw expansion that is microscopically similar to COD
-Can regrow after shave down procedures, can get infected
Describe cemento-ossifying fibroma.
-Benign neoplasm of perio membrane origin
-Asymptomatic swelling and expansion of involved site
-20-40 year olds
-Slight female predilection
-75% mandible, usually in premolar region
-Bowing or convex expansion of the inferior border of the mandible
What are the radiographic/histologic features of cemento-ossifying fibroma.
-Well circumscribed well corticated border
-Can be uni vs multilocular, varying opacity
Histology: Osteoblastic rimming
What is the treatment of cemento-ossifying fibroma?
-Shells out at time of surgery
-Conservative therapy (curettage) usually sufficient
-Rare recurrence
-Malignant transformation to osteosarcoma very uncommon
What are variants of cemento-ossifying fibroma?
-Ossifying fibroma (younger patients, more aggressive)
-Psammomatoid ossifying fibroma (sinonasal tract/orbit)
-Trabecular ossifying fibroma (15M, rapid growing)
Treatment complete surgical excision (curettage-en bloc to segmental resection)
-Recurrence rate 25-58%
Describe central giant cell granuloma
-Younger than 20
-Female>male (2:1)
-Mandible>maxilla
-Affinity to anterior and premolar region
-Will cross midline
-Multilocular
-Giant cells, hypervascular cells
What is the treatment of central giant cell granuloma?
-Small lesions: <5 cm, excision and curettage
-Large lesions: >5 cm, resection or adjunct therapy
-Adjunct therapy: Corticosteroids, calcitonin, interferon alpha
What systemic disease may present similar to a central giant cell granuloma?
-Hyperparathyroidism
Tx the hyperparathyroidism, control renal disease
Describe cherubism.
-Bilateral symmetric expansion of the mandible affecting young children
-AD, SH3BP2 gene mutation on 4p16.3
-Bilateral multilocular radiolucent areas
-Histology identical to CGCG
What is the treatment of cherubism?
-Self limiting by age 30, then cosmetic recontouring after skeletal maturity is reached
Describe aneurysmal bone cyst
-Cystic expansile osteolytic neoplasm
-Blood filled spaces supported by fibrous septa
-Most common in posterior mandible
-Young patient 1-2nd decade
-M=F
-Painful
What is the treatment for an aneurysmal bone cyst?
-Curettage for non-aggressive lesions, resection for large aggressive or recurrent lesions
