Pathology Flashcards

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
Q

What is the histology and IHC of CEOT?

A

-Irregular sheets of islands, amyloid (congo red), ring calcifications
-Can look like carcinoma
-IHS: +Cytokeratin, amyloid, sometimes S100

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26
Q

What is the treatment of CEOT?

A

-Complete surgical excision/thorough curettage/en bloc resection
>20% recurrence rate

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27
Q

What are the variants of CEOT?

A

-Peripheral CEOT (gingiva w/o bone usually anterior jaws)
-Clear cell and pseudoglandular architecture (older pt, 6% of cases)
-6% associated with AOT

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28
Q

What are the clinical features of adenomatoid odontogenic tumor (AOT)?

A

-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

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29
Q

What are the radiographic features of AOT?

A

Well circumscribed radiolucency, 75% associated with unerupted tooth
Radiographic flecks may be present

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30
Q

What is the histology of AOT?

A

-Whirled spindled round odontogenic cells, PAS+, thick fibrous capsule, may co-exist with CEOT

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31
Q

What is the treatment of AOT?

A

Enucleation and curettage, no recurrence

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32
Q

What are the clinical features of ameloblastic fibroma?

A

-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

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33
Q

What are the radiographic features of ameloblastic fibroma?

A

-Well demarcated uni/multi-locular radiolucency, often expansile
-Often associated with unerupted tooth

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34
Q

What is the histology of ameloblastic fibroma?

A

Thin strands/cords of ameloblastic epithelium w/o stellate reticulum
Also contains odontogenic mesenchyme
+Vimentin and ghost cells

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35
Q

What is the treatment of ameloblastic fibroma?

A

Complete excision/en bloc resection (simple curettage is insufficient)
-15-20 % recurrence rate

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36
Q

What are variants of ameloblastic fibroma?

A

Ameloblastic fibrosarcoma (malignant)
-Either de novo or transformation of ameloblastic fibroma

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37
Q

What are the clinical features of primordial odontogenic tumor?

A

-Very rare (less than 30 cases)
-Usually young patients
-Around crown of an impacted tooth

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38
Q

What are the radiographic features of a primordial odontogenic tumor?

A

-Well circumscribed pericoronal radiolucency
-Often associated with an unerupted tooth or in place of a tooth

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39
Q

What is the histology of a primordial odontogenic tumor?

A

-Dental papilla like tissue cuboid-columnar epithelium
-No dental hard tissue

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40
Q

What is the treatment of primordial odontogenic tumor?

A

-Limited data, conservative but complete enucleation

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41
Q

What are the clinical features of an odontoma (includes ameloblastic fibro-odontoma and ameloblastic fibrodentinoma)

A

-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

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42
Q

What are the radiographic features of an odontoma?

A

-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

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43
Q

What is the histology of an odontoma?

A

-All components of odontogenesis present
-Either well formed tooth structure (compound) or haphazard arrangement of products (complex)

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44
Q

What is the treatment of an odontoma?

A

Conservative surgery of enucleation

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45
Q

Describe the clinical features of ameloblastic fibro-odontoma?

A

Variant of odontoma
-Age 10-12 years, 60% under 10
-No sex predilection
-60% mandible, 40% maxilla
-Expansile, asymptomatic

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46
Q

What is the radiographic/histology of a ameloblastic fibro-odontoma?

A

-Well circumscribed mixed density
-Strands of islands and cords, looks a little like odontoma

-Variant of ameloblastic fibrodentinoma contains dentin like material

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47
Q

What is the treatment for ameloblastic fibro-odontoma?

A

Conservative surgery with curettage

48
Q

Describe the clinical features of dentinogenic ghost cell tumor

A

-20-70 years old
-May present as non-healing extraction site, jaw expansion, painful swelling or ulcerated mass
-Extraosseous are less aggressive

49
Q

What are radiographic signs of dentinogenic ghost cell tumor?

A

-Irregular, destructive mixed lesion
-May have opacification of sinus
-Solid neoplastic version of a COC (Calcifying odontogenic cyst)

50
Q

What is the histology of dentinogenic ghost cell tumor?

A

-Resembles ameloblastoma in association with COC, ghost cells and dystrophic mineralization

51
Q

What is the treatment of dentinogenic ghost cell tumor?

A

-Wide surgical resection; may metastasize

52
Q

What are the clinical features of odontogenic fibroma?

A

-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
Q

What are the radiographic features of odontogenic fibroma?

A

-Well demarcated radiolucency, usually at root of tooth
-Displacement, resorption may be present

54
Q

What is the histology of odontogenic fibroma?

A

-Proliferation of bland fibrous tissue and scattered rests of odontogenic epithelium

-Differential: hyperplastic dental follicle, desmoplastic fibroma of bone, infantile myofibromatosis

55
Q

What is the treatment of odontogenic fibroma?

A

-Conservative local excision or curettage is sufficient

56
Q

What are the clinical features of odontogenic myxoma?

A

-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
Q

What are the radiographic signs of odontogenic myxoma?

A

-Multilocular, well demarcated, can be ill defined
-Can have honeycomb trabecular pattern
-Root displacement not resorption

58
Q

What are the histologic properties of a myxoma?

A

-Dental papilla, delicate myxoid connective tissue, +vimentin, +S100

59
Q

What is the treatment of odontogenic myxoma?

A

-Aggressive odontogenic tumor
-Recurrence around 25%
-Resection with 1-1.5 cm margins and anatomic barrier

60
Q

What are the clinical features of cementoblastoma?

A

-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
Q

What are the radiographic features of cementoblastoma?

A

-Well defined radiopaque mass adhered to the root with a thin radiolucent halo, no PDL seen between lesion and root

62
Q

What is the histology of cementoblastoma?

A

-Dense basophilic, cementum like tissue

63
Q

What is the treatment of cementoblastoma?

A

-Surgical removal of tooth and lesion
-Need to completely remove lesion or can recur

64
Q

What are the clinical features of ameloblastic carcinoma?

A

-Histologic/behavior of malignant ameloblastic neoplasm, no calcified material
-Mean age 30
-Mets to lung (75%) or lymph nodes/spine (15%)

65
Q

What are the radiographic features of ameloblastic carcinoma?

A

-Aggressive ill-defined radiolucency
-May expand the cortical plates, erode them and resorb roots

66
Q

What is the histology of ameloblastic carcinoma?

A

-Atypia and malignancy in neoplastic ameloblastic epithelium
-No dentin, enamel, or cementum

67
Q

What is the treatment of ameloblastic carcinoma?

A

Staged and treated as carcinoma involving the jaws

68
Q

What are the clinical features of a dentigerous cyst?

A

-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
Q

What is the treatment for a dentigerous cyst?

A

Surgical removal (may recur), removal of impacted tooth, may give rise to ameloblastoma, SCCa, mucoepidermoid carcinoma

70
Q

What are the variants of a dentigerous cyst?

A

Eruption cyst, primordial cyst

71
Q

What are the clinical features of an OKC?

A

-Any age, mostly 2-3rd decade
-1/2 are symptomatic
-More common in mandible posterior area
-High recurrence

72
Q

What are the radiographic features of an OKC?

A

75% unilocular (25% multi-locular)
-May have tooth displacement or resorption

73
Q

What is the histology of an OKC?

A

Corrugated parakeratinized stratified squamous epithelium, 6-8 cells thick, palisaded basal layer

74
Q

What is the treatment for OKC?

A

Conservative to aggressive surgical removal
-Peripheral ostectomy depending on anatomy
-Recurrence high due to incomplete removal, satellite cysts

75
Q

What is nevoid basal cell carcinoma syndrome?

A

-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
Q

What is the treatment of lateral periodontal cyst/botryoid odontogenic cyst?

A

-Surgical excision
-Botryoid more likely to recur

77
Q

What is gingival cyst of infants?

A

-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
Q

What are the features of glandular odontogenic cyst (clinical, radiographic, histology)?

A

-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
Q

What is the treatment of glandular odontogenic cyst?

A

-Wide local exicion/resection due to possibility of recurrences

80
Q

What are the clinical/raiographic/histological features of calcifying odontogenic cyst?

A

-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
Q

What is the treatment for calcifying odontogenic cyst?

A

Surgical excision
-May have recurrence or malignant transformation
-If associated with aggressive odontogenic tumor or cyst, treated as a neoplasm

82
Q

Describe orthokeratinized odontogenic cyst?

A

-Not the same as OKC
-Less likely to recur than OKC
-Male predilection

-Surgical removal

83
Q

Describe a radicular/periapical cyst.

A

-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
Q

Describe clinical features of a chondrosarcoma.

A

-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
Q

What is the treatment of chondrosarcoma?

A

-Wide excision with clean margins if possible

-Histologic grade and clear margins most predictive factors

-20% 5 year survival

86
Q

What are the features of mesenchymal chondrosarcoma?

A

-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
Q

What is the treatment of mesenchymal chondrosarcoma?

A

-Complete resection

-Relatively good prognosis if completely resected, late metastases are seen requiring long term follow-up

88
Q

What are the features of osteosarcoma?

A

-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
Q

What other processes are associated with increased incidence of osteosarcoma?

A

-Li Fraumeni syndrome, retinoblastoma, Werner syndrome, Rothmond-Thompson syndrome, giant cell tumor, chronic osteomyelitis, OI

90
Q

Describe histological findings with osteosarcoma.

A

-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
Q

Describe the treatment of osteosarcoma.

A

-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
Q

Describe Paget’s disease of bone.

A

-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
Q

Describe fibrous dysplasia

A

-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
Q

What are the types of fibrous dysplasia?

A

Monostotic
Craniofacial
Polyostotic

95
Q

What is monostotic fibrous dysplasia?

A

70% of cases involving a single bone
-Jaws, ribs, femur and tibia most common sites
-Young adults

96
Q

What is craniofacial fibrous dysplasia?

A

-Slightly younger age group
-Usually more than one bone but can be mandible only
-No endocrine dysfuntion

97
Q

What is polyostotic fibrous dysplasia?

A

-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
Q

What is McCune Albright and Jaffe’s syndrome?

A

McCune Albright: Polyostotic fibrous dysplasia with cafe au lait spots, endocrine abnormalities (precocious anemia)

Jaffe’s syndrome: Polyostotic fibrous dysplasia with skin pigmentation

99
Q

What are radiographic/histologic features of fibrous dysplasia?

A

-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
Q

What is the treatment of fibrous dysplasia?

A

-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
Q

Describe cemento-osseous dysplasia

A

-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
Q

What is the radiographic/histologic features of cemento-osseous dysplasia?

A

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
Q

What is the treatment of cemento-osseous dysplasia?

A

-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
Q

What is familial giantiform cementoma?

A

-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
Q

Describe cemento-ossifying fibroma.

A

-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
Q

What are the radiographic/histologic features of cemento-ossifying fibroma.

A

-Well circumscribed well corticated border
-Can be uni vs multilocular, varying opacity

Histology: Osteoblastic rimming

107
Q

What is the treatment of cemento-ossifying fibroma?

A

-Shells out at time of surgery
-Conservative therapy (curettage) usually sufficient
-Rare recurrence
-Malignant transformation to osteosarcoma very uncommon

108
Q

What are variants of cemento-ossifying fibroma?

A

-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
Q

Describe central giant cell granuloma

A

-Younger than 20
-Female>male (2:1)
-Mandible>maxilla
-Affinity to anterior and premolar region
-Will cross midline

-Multilocular

-Giant cells, hypervascular cells

110
Q

What is the treatment of central giant cell granuloma?

A

-Small lesions: <5 cm, excision and curettage
-Large lesions: >5 cm, resection or adjunct therapy

-Adjunct therapy: Corticosteroids, calcitonin, interferon alpha

111
Q

What systemic disease may present similar to a central giant cell granuloma?

A

-Hyperparathyroidism

Tx the hyperparathyroidism, control renal disease

112
Q

Describe cherubism.

A

-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
Q

What is the treatment of cherubism?

A

-Self limiting by age 30, then cosmetic recontouring after skeletal maturity is reached

114
Q

Describe aneurysmal bone cyst

A

-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
Q

What is the treatment for an aneurysmal bone cyst?

A

-Curettage for non-aggressive lesions, resection for large aggressive or recurrent lesions