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Pathology Flashcards

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1
Q

What are epithelial origin benign tumors?

A

-Ameloblastoma (Conventional solic, multicystic, unicystic, extraosseous/peripheral)

-Squamous odontogenic tumor

-Calcifying epithelial odontogenic tumor

-Adenomatoid odontogenic tumor

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2
Q

What are mixed (epithelial and mesenchymal) origin tumors?

A

-Ameloblastic fibroma

-Primordial odontogenic tumor

-Odontoma (compound, complex, dentinogenic ghost cell tumor)

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3
Q

What are mesenchymal origin tumors?

A

-Odontogenic fibroma

-Odontogenic myxoma

-Cementoblastoma

-Cemento-ossifying fibroma

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4
Q

What are malignant odontogenic tumors?

A

-Ameloblastic carcinoma
-Primary intraosseous carcinoma, not otherwise specified
-Sclerosing odontogenic carcinoma
-Clear cell odontogenic carcinoma
-Odontogenic carcinosarcoma
-Odontogenic sarcoma

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5
Q

What are the types of odontogenic cysts?

A

-Developmental: Dentigerous, OKC, lateral perio/botryoid cyst, gingival cyst, glandular odontogenic cyst, calcifying odontogenic cyst, orthokeratinized odontogenic cyst

-Inflammatory: Radicular, collateral inflammatory cyst

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6
Q

Describe ameloblastoma

A

Three types:
-Conventional (solid, multi-cystic)
-Unicystic
-Extra-osseous (peripheral)

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7
Q

What age group do you expect to see ameloblastoma?

A

Occur at any age.

50% of all cases are between 20-40 years old

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8
Q

What demographic do you see ameloblastoma?

A

No sex predilection

20-40 year old

80% in mandible (75% molar/ramus region)
20% in maxilla (mostly posterior)

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9
Q

What are some pre-op features that can present with ameloblastoma?

A

Slow growing, expansile odontogenic epithelial neoplasm

See dental changes: Mobility, displacement, root resorption

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10
Q

What are radiographic features of ameloblastoma?

A

Multilocular radiolucency (can also be unilocular

20% associated with unerupted tooth

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11
Q

What is the histology of ameloblastoma?

A

-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)

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12
Q

What is the treatment of ameloblastoma?

A

-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.

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13
Q

What is the cure and recurrence rate of ameloblastoma?

A

98% cure from resection

Recurrence of 70-85% for enucleation and curettage due to incomplete removal

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14
Q

What is desmoplastic ameloblastoma?

A

-Older group, anterior jaw, maxilla>mandible

-Radiographs suggest fibro-osseous lesion

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15
Q

What is unicystic ameloblastoma?

A

-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)

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16
Q

What is peripheral ameloblastoma?

A

-Arising from oral surface epithelium (gingival)
-Not aggressive, does not invade bone
-Low recurrence rate

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17
Q

What is sinonasal ameloblastoma?

A

-Males, 61-year olds, from pluripotential cells of sinonasal epithelium
-Presents with nasal obstruction and epistaxis
-Usuallh plexiform, no metastasis

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18
Q

What are the molecular features of ameloblastoma?

A

-Mutations of MAPK pathway in 90% of ameloblastomas.
-BRAF-gargeted therapy in aggressive/recurrent ameloblastomas

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19
Q

What are the clinical features of squamous odontogenic tumor?

A

-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

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20
Q

What are the radiographic features of squamous odontogenic tumor?

A

-Well defined semicircular or triangular radiolucent defect around the roots of affected teeth, similar appearance t juvenile perio and eosinophilic granuloma

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21
Q

What is the histology of squamous odontogenic tumor?

A

-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+)

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22
Q

What is the treatment of squamous odontogenic tumor?

A

Complete surgical excision through curettage, recurrence has been reported

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23
Q

What are the clinical features of calcifying odontogenic tumor?

A

-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

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24
Q

What are the radiographic features of CEOT?

A

-Variable. uni vs multilocular radiolucency. Mixed ensity lesion, often with an unerupted tooth, eventual expansion of the cortical plates

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25
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
26
What is the treatment of CEOT?
-Complete surgical excision/thorough curettage/en bloc resection >20% recurrence rate
27
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
28
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
29
What are the radiographic features of AOT?
Well circumscribed radiolucency, 75% associated with unerupted tooth Radiographic flecks may be present
30
What is the histology of AOT?
-Whirled spindled round odontogenic cells, PAS+, thick fibrous capsule, may co-exist with CEOT
31
What is the treatment of AOT?
Enucleation and curettage, no recurrence
32
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
33
What are the radiographic features of ameloblastic fibroma?
-Well demarcated uni/multi-locular radiolucency, often expansile -Often associated with unerupted tooth
34
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
35
What is the treatment of ameloblastic fibroma?
Complete excision/en bloc resection (simple curettage is insufficient) -15-20 % recurrence rate
36
What are variants of ameloblastic fibroma?
Ameloblastic fibrosarcoma (malignant) -Either de novo or transformation of ameloblastic fibroma
37
What are the clinical features of primordial odontogenic tumor?
-Very rare (less than 30 cases) -Usually young patients -Around crown of an impacted tooth
38
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
39
What is the histology of a primordial odontogenic tumor?
-Dental papilla like tissue cuboid-columnar epithelium -No dental hard tissue
40
What is the treatment of primordial odontogenic tumor?
-Limited data, conservative but complete enucleation
41
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
42
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
43
What is the histology of an odontoma?
-All components of odontogenesis present -Either well formed tooth structure (compound) or haphazard arrangement of products (complex)
44
What is the treatment of an odontoma?
Conservative surgery of enucleation
45
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
46
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
47
What is the treatment for ameloblastic fibro-odontoma?
Conservative surgery with curettage
48
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
49
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)
50
What is the histology of dentinogenic ghost cell tumor?
-Resembles ameloblastoma in association with COC, ghost cells and dystrophic mineralization
51
What is the treatment of dentinogenic ghost cell tumor?
-Wide surgical resection; may metastasize
52
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
53
What are the radiographic features of odontogenic fibroma?
-Well demarcated radiolucency, usually at root of tooth -Displacement, resorption may be present
54
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
55
What is the treatment of odontogenic fibroma?
-Conservative local excision or curettage is sufficient
56
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
57
What are the radiographic signs of odontogenic myxoma?
-Multilocular, well demarcated, can be ill defined -Can have honeycomb trabecular pattern -Root displacement not resorption
58
What are the histologic properties of a myxoma?
-Dental papilla, delicate myxoid connective tissue, +vimentin, +S100
59
What is the treatment of odontogenic myxoma?
-Aggressive odontogenic tumor -Recurrence around 25% -Resection with 1-1.5 cm margins and anatomic barrier
60
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
61
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
62
What is the histology of cementoblastoma?
-Dense basophilic, cementum like tissue
63
What is the treatment of cementoblastoma?
-Surgical removal of tooth and lesion -Need to completely remove lesion or can recur
64
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%)
65
What are the radiographic features of ameloblastic carcinoma?
-Aggressive ill-defined radiolucency -May expand the cortical plates, erode them and resorb roots
66
What is the histology of ameloblastic carcinoma?
-Atypia and malignancy in neoplastic ameloblastic epithelium -No dentin, enamel, or cementum
67
What is the treatment of ameloblastic carcinoma?
Staged and treated as carcinoma involving the jaws
68
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
69
What is the treatment for a dentigerous cyst?
Surgical removal (may recur), removal of impacted tooth, may give rise to ameloblastoma, SCCa, mucoepidermoid carcinoma
70
What are the variants of a dentigerous cyst?
Eruption cyst, primordial cyst
71
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
72
What are the radiographic features of an OKC?
75% unilocular (25% multi-locular) -May have tooth displacement or resorption
73
What is the histology of an OKC?
Corrugated parakeratinized stratified squamous epithelium, 6-8 cells thick, palisaded basal layer
74
What is the treatment for OKC?
Conservative to aggressive surgical removal -Peripheral ostectomy depending on anatomy -Recurrence high due to incomplete removal, satellite cysts
75
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
76
What is the treatment of lateral periodontal cyst/botryoid odontogenic cyst?
-Surgical excision -Botryoid more likely to recur
77
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
78
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
79
What is the treatment of glandular odontogenic cyst?
-Wide local exicion/resection due to possibility of recurrences
80
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
81
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
82
Describe orthokeratinized odontogenic cyst?
-Not the same as OKC -Less likely to recur than OKC -Male predilection -Surgical removal
83
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
84
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
85
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
86
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
87
What is the treatment of mesenchymal chondrosarcoma?
-Complete resection -Relatively good prognosis if completely resected, late metastases are seen requiring long term follow-up
88
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
89
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
90
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
91
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
92
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
93
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
94
What are the types of fibrous dysplasia?
Monostotic Craniofacial Polyostotic
95
What is monostotic fibrous dysplasia?
70% of cases involving a single bone -Jaws, ribs, femur and tibia most common sites -Young adults
96
What is craniofacial fibrous dysplasia?
-Slightly younger age group -Usually more than one bone but can be mandible only -No endocrine dysfuntion
97
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)
98
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
99
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
100
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
101
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
102
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)
103
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
104
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
105
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
106
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
107
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
108
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%
109
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
110
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
111
What systemic disease may present similar to a central giant cell granuloma?
-Hyperparathyroidism Tx the hyperparathyroidism, control renal disease
112
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
113
What is the treatment of cherubism?
-Self limiting by age 30, then cosmetic recontouring after skeletal maturity is reached
114
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
115
What is the treatment for an aneurysmal bone cyst?
-Curettage for non-aggressive lesions, resection for large aggressive or recurrent lesions

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