JAW BONE LESIONS ODONTOGENIC TUMORS

sompid/Jaw bone lesions II 1

JAW BONE LESIONSODONTOGENIC TUMORS

Dr. Sompid Kintarak

Department of Stomatology

Prince of Songkla University

Tooth development

Classification

1. Benign odontogenic tumorA. Tumor of odontogenic epithelium with mature,

fibrous stroma without odontogenic ectomesenchyme

B. Mixed odontogenic tumor: composed of odontogenic epithelium and odontogenic ectomesenchyme, with or without hard tissue formation

C. Tumor of odontogenic ectomesenchyme with or without odontogenic epithelium

2. Malignant odontogenic tumorA. Odontogenic carcinomaB. Odontogenic sarcoma

Tumor of odontogenic epithelium with mature, fibrous stroma without odontogenic ectomesenchyme

1. Ameloblastoma

- solid/multicystic type

- desmoplastic type

- unicystic type

- extraosseous / peripheral type

2. Squamous odontogenic tumor

3. Calcifying epithelial odontogenic tumor

4. Adenomatoid odontogenic tumor

5. Keratocystic odontogenic tumor

Tumor of odontogenic epithelium with odontogenic ectomesenchyme, with or without hard tissue formation

1. Ameloblastic fibroma

2. Ameloblastic fibro-odontoma

3. Compound / Complex odontoma

4. Odontoameloblastoma

5. Calcifying cystic odontogenic tumor

6. Dentinogenic ghost cell tumor

Tumor of odontogenic ectomesenchyme with or without odontogenic epithelium

1. Odontogenic fibroma

2. Granular cell odontogenic tumor

3. Odontogenic myxoma

4. Cementoblastoma


Odontogenic carcinomas

1. Metastasizing (malignant) ameloblastoma

2. Ameloblastic carcinoma

– primary type

– secondary type (dedifferentiated), intraosseous

– secondary type (dedifferentiated), peripheral

3. Primary intraosseous squamous cell carcinoma

– solid type

- derived from keratocystic odontogenic tumor

- derived from odontogenic cysts

4. Clear cell odontogenic carcinoma

5. Ghost cell odontogenic carcinoma

Odontogenic sarcomas

1. Ameloblastic fibrosarcoma

2. Ameloblastic fibro-odontosarcoma

Ameloblastoma

1. Conventional solid or multicystic intraosseous ameloblastoma (~86%)

2. Desmoplastic ameloblastoma (3%-13% of intra-osseous ameloblastoma)

3. Unicystic ameloblastoma (~13%)4. Peripheral (extraosseous) ameloblastoma (~1%)

Conventional ameloblastoma

Wide age range 30-70 y; mean 28.7 y in blacks, 39.9 y in Caucasians, 41.2 y in Asiansuncommon under 20 and rare under 10 yM ~ F85% in mandible: molar-ascending ramus15% in maxilla: posterior regionsDesmoplastic type: anterior part of the jawspainless swelling or expansion of the jawmay grow slowly to a very large sizepain and paresthesia are uncommon even in large tumor

Ameloblastoma 486 cases from Dentistry, CU (J Dent Assoc Thai 2003;53:349-356)

• Peak 20 to 39 y (46.3%), mean 34.37 y

• M = F

• mandible (89.5%), premolar-molar region

• maxilla (9.3%), anterior-premolar region

• swelling (9.4%), pain (16.5%), tooth mobility (25.7%), pus discharge (9.7%), paresthesia (6.4%)

• Multilocular (64.7%), unilocular (30.9%)

• solid or multicystic type (88.5%), unicystic (11.5%)

• plexiform and/or follicular pattern (59.7%)

Conventional ameloblastoma

X-ray: multilocular radiolucent lesion

‘soap bubble’ appearance

‘honeycombed’ appearance

buccal & lingual cortical expansion

root resorption of adjacent teeth is common

may associated with an unerupted tooth, most often lower third molars

may appear as unilocular radiolucence often with irregular scalloped borders


Ameloblastoma

Microscopic patterns of ameloblastoma

Follicular - most common

Plexiform

Acanthomatous

Granular cell

Basal cell

Follicular ameloblastoma (tumor epithelial islands in a mature fibrous connective stroma)

the epithelial islands consist of a core of angular cells, resembling stellate reticulum of an enamel organ, surrounded by a well-organized single layer (palisaded) of tall columnar ameloblast-like cells with nuclei at the opposite pole to the basement membrane (reversed nuclear polarity) and cytoplasmic vacuolization cyst formation within the epithelial islands

Plexiform ameloblastoma(thin trabeculae or long anastomosing cords or larger sheets of tumor epithelium in a loosely arranged and vascularized fibrous connective stroma)

the cords of epithelium consist of a core of ameloblast-like cells (less-well-differentiated than the ameloblast-like cells in follicular type) surrounding more loosely arranged epithelial cellscyst formation, if form, is in the stroma (stromal degeneration)

Acanthomatous type: squamous metaplasia with keratin formation in the central portions of the epithelial islands of a follicular ameloblastoma

Granular cell pattern: resemble follicular type but the tumor epithelial cells show transformation to granular cellsgranular cells are large and contain abundant eosinophilic granules in the cytoplasm

Basal cell pattern: solid islands or nests of uniform basaloid cells in fibrous stroma

• resemble basal cell carcinoma of the skin

• nests of darkly stained basaloid cells surrounded by cuboidal cells

• no stellate reticulum in the epithelial nests


Treatment of ameloblastoma

Marginal resection including up to 1.5 cm of clinically normal bone around the margin or at least 1 cm past the radiographic limits of the tumor

radical excision (maxillectomy) of maxillary ameloblastoma at the earliest possible stage is essential; effective re-operation is unlikely to be possible

5-yr disease free periods do not indicate a cure

Unicystic ameloblastoma

Younger than conventional ameloblastomamean age 23 y.mandible, posterior regionsclinical resembling a dentigerous cyst, radicular cyst or residual cystX-ray: unilocular, well-defined radiolucent lesion

may have scalloped margins

Unicystic ameloblastoma

luminal unicystic

intraluminal unicystic

or plexiform unicystic

mural unicystic

Treatment of unicystic ameloblastoma

luminal and intraluminal types

- conservative surgical removal / enucleation

- long-term follow-up

- low recurrence rate (10%-20%)

mural type

- resection as in conventional ameloblastomas

Desmoplastic ameloblastoma

Small, irregular islands of tumor epithelium in a dense collagenous fibrous tissue~3%-13% of intraosseous ameloblastomapredilection for the anterior regionsX-ray: unilocular or multilocular, usually ill-defined mixed radiolucent and radiopaque lesion ~ fibro-osseous lesionameloblast-like cells are only present in small foci on the periphery of some islands of epitheliumno cyst formation and stellate reticulum-like tissue is absent

X48-94

Increased production of TGF-beta that may be responsible for desmoplasia


Peripheral ameloblastoma

May arise from rests of dental lamina beneath the oral mucosa, or basal epithelial cells of the surface epitheliummiddle-aged 40-60 y patients, average 50 y.mandibular gingiva (66%), premolar regionmaxilla, tuberosity areapainless, slow-growing, sessile or pedunculated gingival mass, irregular surfacemost are < 1.5 cmlittle or no bone erosion (saucerization)Clinical DDX: fibrous nodule, pyogenic granuloma, other gingival lesions

Peripheral ameloblastoma. Hercellio et al. OOOOE 2005;99:E31-3

20M asymptomatic slow growing sessile 2.0-cm pink lesion on the right posterior lingual gingiva. Presence for 4 months. No significant radiographic findings.

Peripheral ameloblastoma

Histo: all types of ameloblastoma have been reported in the peripheral lesion

Treatment: local excisionrecurrence rate ~9%wider local excision for the recurrence lesion

Malignant ameloblastoma

Tumor that shows typical histopathologic features of ameloblastoma, in both the primary and metastatic lesions

Primary tumor ~88.5% in mandible

mean age 27.9 y, 33% age < 20 y

Time to metastasis 2-31 y (mean 11 y)

Malignant ameloblastoma

88% pulmonary metastasis (intrapleural, hilar node)

29% cervical LN metastases, 19% each to pleura and bone

Other sites: diaphragm, liver, small intestine, brain, kidney

by definition, diagnosis can only be made after it has metastasized

Tx: too few cases for a definitive treatment protocol, however, treatment of choice is excision

median duration of survival after treatment of the metastasis was 2-3 years

Ameloblastic carcinoma

Tumor that shows cytologic features of malignancy but is otherwise recognizable as an ameloblastomaClinic: pain, rapid-growing-swelling of the jaw; age 4-84 (mean 37.2 y); mandible (75%) at posterior, trismus, paresthesia, tooth mobility, dysphagia

X-ray: ill-defined radiolucency, root resorption, buccal and lingual bone expansion and perforation, extension to the soft tissues

Tx: wide excision

poor prognosis


Ameloblastic carcinoma: rapidly growing tumor with buccal expansion of the left mandible showing irregular destruction of the mandible.

Squamous odontogenic tumor

Rare tumor (< 50 cases)First described by Pullon et al in 1975may arise from dental lamina, or rests of Malasszeappears to originate within the periodontal ligament associated with the lateral root surface of an erupted tooth

Squamous odontogenic tumor (SOT)

Clinic: mainly involve the alveolar process close to the roots of erupted teeth, which they may loosen

– painless swelling *may be mistaken for periodontal disease

– ~25% no symptoms, detected by X-ray

X-ray: non-specific

- can mimic severe bone loss ~periodontal disease

- cyst-like area; most are < 1.5 cm in diameter

Histo: varying-shaped islands of bland-appearing squamous epithelium in a mature (moderately cellular) fibrous connective tissue stroma

– keratin, calcification, globular eosinophilic structures, or microcysts may form within the islands

– may show continuation with the surface epithelium

Tx: curettage or conservative excision and extraction of any involved teeth

- maxillary SOT may be more aggressive than mandibular lesion

Calcifying epithelial odontogenic tumor

Pindborg tumor (described by Pindborg in 1958)~ 1% of odontogenic tumors~88% intraosseous; age 30-50 ymandible (80%): premolar-molar regiona painless, slow-growing swelling

X-ray: multilocular or honeycombed radiolucencymay be well-defined or more poorly defined (~20%)

often unilocular in early stagediffused radiopaque masses of variable sizeoften associated with an impacted mandibular third molar


CEOT

Histo: sheets or strands of pleomorphic, slightly eosinophilic epithelial cells in a connective tissue stroma– gross variation in nuclear size

– hyperchromatic nuclei and multinucleated cells

intercellular bridge may be clearly seen

large area of amyloid-like extracellular material in tumor [amorphous, eosinophilic, homogeneous or hyalinized material]

calcification of amyloid-like areas in a concentric rings (Liese-gang ring calcifications)

~ 6% clear-cell variant: DDX from clear cell odontogenic carcinoma

CEOT

Behavior: not encapsulated, locally invasive but little less aggressive than ameloblastoma

Treatment: conservative local resection with a narrow rim of surrounding normal bone

recurrence rate ~15%

CEOT: from Lucas’s pathology, 5th ed. And Neville’s pathology, 2nd ed.

Adenomatoid odontogenic tumor

represent 3%-7% of odontogenic tumor

common in 10-19 y (66%); uncommon after 30 y

Female predilection, anterior maxilla

very slow-growing, painless swelling

peripheral lesions are rare

X-ray: well-defined cyst-like radiolucency, <3.0 cm

60% contain radiopaque foci (snow flake)

75% associated with unerupted tooth (upper canine) ~dentigerous cyst, lateral periodontal cyst, or may enclose part of root


Adenomatoid odontogenic tumor: well-defined thick capsule with central portion of tumor (solid or cystic) - Duct-like microcysts lined by ameloblast-like cells among whorl masses of spindle-shaped epithelial cells in a scant stroma- Small foci of calcification are scattered throughout the tumor (abortive enamel formation), occasionally dentinoid or tubular dentine is formed

Ameloblastic fibromaSome histological resemblance to ameloblastoma, but are more benign in behavior and are more uncommonmean age 15 ymandible (79%): bicuspid-molar areaslow growing, asymptomatic, bone expansion later- 58% swelling- 17% detected from routine X-

ray

Radiograph: multilocular radiolucency with sclerotic borders, 1-8 cm in diameter, small lesions may appear unilocular- associated erupted tooth (75%)

Microscopic features of ameloblastic fibroma

Odontogenic epithelium: islands, strands or cauliflower-like proliferation of tumor epithelium showing ameloblast-like cells surrounding stellate reticulum-like structure

- long, narrow cords (two cell thick) of odontogenic epithelium

Mesenchymal component: resembling primitive dental papilla - a loose but cellular (plump stellate and ovoid cells) fibromyxoid connective tissue

Treatment of ameloblastic Fibroma

Conservative initial therapy and more aggressive surgical excision for recurrent lesionshigh recurrence (43.5%) with simple local excision or curettage~45% of ameloblastic fibrosarcoma cases develop from recurrence ameloblastic fibromaprolonged follow-up

Ameloblastic fibro-odontomaAmeloblastic fibroma + odontomayounger age than AF; mean age 8-10 yposterior regionClinic: asymptomatic, found on X-ray to determine for failure of a tooth to erupt or painless swelling

Radiograph: – a well-circumscribed unilocular – or, rarely, multilocular radiolucency containing calcifications having the radiodensity of tooth structure

Ameloblastic fibro-odontoma: ameloblastic fibroma with toothlike structures containing enamel, dentine masses

Ameloblastic fibrodentinoma: ameloblastic fibroma with only dentine matrix, dentinoid material

Treatment:– Conservative currettage (easily removed from its bony bed)

– Excellent prognosis


Ameloblastic fibrosarcomaAmeloblastic sarcomaLow-grade, locally infiltrative sarcoma

~50% of cases develop in ameloblastic fibroma or ameloblastic fibro-odontoma

mean age 27.5 y; M > F (1.5:1); mandible > maxilla

Clinic: rapid-growing, pain, swelling, locally aggressive and infiltrates adjacent bone and soft tissues

X-ray: ill-defined destructive radiolucent lesion

Histo: malignant features of the menchymal component

– highly cellular

– pleomorphic, hyperchromatic cells

– prominent mitoses

– may contain enamel or dysplastic dentine

Treatment:

– radical surgical excision

– most deaths have been resulted from uncontrolled local disease

– metastatic tumor has been reported in 3/53 cases [different from fibrosarcoma – 25% metastasis]

Odontoameloblastoma

Ameloblastoma + Odontoma (compound or complex type)

rare (~0.5%) odontogenic tumors

same biologic potential as the ameloblastoma

Odontoma

Odontoma

The most common type of odontogenic tumors

considered to be hamartoma rather than true neoplasm

consists of enamel, dentin with variable amounts of pulp and cementum, and varying amounts of proliferating odontogenic epithelium and mesenchyme

2 types: compound (composed of multiple, small tooth like structures) – anterior maxilla

complex (irregular mass of hard and soft dental tissues, having no tooth morphology) –posterior region of either jaw

Mean age 14 y

may found in the gingival soft tissues

mostly asymptomatic, small lesions, diagnosed from routine X-ray

large lesions can cause jaw bone expansion

X-ray: often associated with unerupted tooth


Calcifying cystic odontogenic tumorCalcifying odontogenic cyst

Calcifying cystic odontogenic tumor

Ghost epithelial cells within the epithelium and dentine-like material adjacent to the epithelial component


Cystic (nonneoplastic) forms comprise 86%-98%

13%-30% of cases are peripheral (extraosseous)

arise from rests of the dental lamina

maxilla = mandible (incisor and canine areas)

wide age range, mean age 33 y.

may be associated with odontogenic tumor eg

- odontoma: 20%-26%, mean age 17 y

- adenomatoid odontogenic tumor

- ameloblastoma

- ameloblastic fibroma


Painless swelling with bone expansion, occasionally perforated0.5-12 cm in diameter, mostly 2-4 cmHas a potential for continued growthX-ray: well-defined, unilocular radiolucency (occasionally multilocular)– scattered radiopacities (33%-50%)– root resorption– divergence of adjacent teeth– associated with unerupted tooth (33%) - canine– associated with odontoma (26% of cases)

Calcifying cystic odontogenic tumor:

Well-defined, unilocular radiolucent lesion in the mandibular premolar-canine area.

Well-defined, unilocular radiolucent lesion of the anterior maxilla with central odontoma.


Treatment of CCOT:– simple enucleation is treatment of choice

– prognosis is good

– more extensive surgery may be required if there is any doubt whether the lesion can be removed in its entirely

– minimal recurrence, however, prolonged follow-up is required

– wide excision for malignant form and careful follow-up is necessary

Dentinogenic ghost cell tumor

Ghost cell odontogenic carcinoma

Solid variant (2%-16%) is considered as neoplasm

Benign: dentinogenic ghost cell tumor

– presence of ameloblastoma-like strands and odontogenic epithelial islands in the fibrous connective tissue stroma, mitosis is not evident

– variable numbers of ghost cells and juxtaepithelial dentinoid

Malignant: ghost cell odontogenic carcinoma (rare)

– 19 reported cases: > 50% were from Asia

– predilection for maxilla, painful expansion of bone

– cellular pleomorphism, presence of mitoses

– presence of ghost cells and dentinoid

– invasion of the surrounding tissues

Peripheral CCOT

13-30% of all CCOT

sessile or pedunculated gingival mass with no distinctive clinical features

usually 0.5-1 cm in diameter

may resemble gingival fibroma, gingival cyst, peripheral giant cell granuloma

mandibular incisor/canine and premolar areas

from rests of Serres

36% are solid type

Tx: simple excision

– prognosis ~ peripheral ameloblastoma

Central odontogenic fibromaAffect tooth-bearing area

4-80 y, mean age 40 y

F > M (2.2:1)

Maxilla (45%) - anterior to the first molar

Mandible - posterior to the first molar

1/3 associated with an unerupted tooth

slow growing lesion

small lesion - asymptomatic

large lesion - localized bone expansion or loosening of teeth

Central odontogenic fibroma: x-ray– well-defined, radiolucent lesions often associated with

the periradicular area of erupted teeth

– unilocular in small lesions

– scalloped borders or multilocular in large lesions

– root resorption is common

– root divergence

– patchy radiopaque in the lesion (~12%)


Central odontogenic Fibroma: histo

normally no definite capsule

Simple type: resembles a dental follicle

– scattered stellate fibroblasts within a collagenous background

– odontogenic epithelial rests may or may not be present

– occasional dystrophic calcification is present

Complex type or WHO type:

– cellular fibrous connective tissue containing odontogenic epithelium in the form of long strands, cords or nests

– the fibrous component may vary from myxoid to densely hyalinized

– dentinoid and cementum-like material may present

– giant cell granuloma-like component may present

Central odontogenic Fibroma

Treatment:

enucleation and vigorous curettage

very good prognosis

Peripheral odontogenic fibromafacial gingiva of the mandible ~fibrous epulis

size 0.5 to 1.5 cm in diameter

may cause displacement of the teeth

Histo: ~WHO type

– Granular cell changes or giant cell granuloma-like areas have been described

Granular cell odontogenic tumor~30 cases have been reported

> 50% are > 40 y; female (70%)

mandible: premolar-molar region

peripheral tumor as gingival mass

X-ray: unilocular or multilocular, well-defined border, occasional calcifications

Histo: large eosinophilic granular mesenchymal cells (negative for S-100) with small nests of odontogenic epithelium; small cementum-like or dystrophic calcifications associated with granular cells in some lesions Ana Terezinha Marques Mesquita, Cássio Roberto Rocha Santos, Ricardo

Santiago Gomez, Jacks Jorge, Jorge Esquiche León, Oslei Paes de Almeida. Central granular cell odontogenic tumor: a histopathologic and immunohistochemical study. Annals of Diagnostic Pathology 2009;13:405-412.

Odontogenic myxomaDerived from odontogenic mesenchyme

locally destructive lesion, tooth-bearing area

young adults, mean age 25-30 y

rare in <10 y or >50 y

slow-growing, painless swelling of the jaw

unilocular or multilocular radiolucency

– “soap bubble” appearance ~ ameloblastoma

– occasional loosening, displacement, or resorption of teeth

Odontogenic myxoma

Gross specimen: gelatinous, loose structureHisto: – stellate, spindle-shaped cells with long processes, and round cells in loose myxomatous stroma

– may contain small, scattered epithelial rests– no capsule, tend to infiltrate the surrounding bone– when formation of more collagen - fibromyxoma


Odontogenic myxoma: treatment

small lesions - curettage, follow-up at least 5 yearslarge lesions - more extensive resectionrecurrence ~25%

Myxosarcomamarked cellularity & cellular atypismlocal aggressiveno reported metastasis

Cementoblastoma (true cementoma)

Cementoblastoma

Unlimited growth potential

histologic features identical to osteoblastoma

mandible (75%), premolar-molar region (90%)

~50% involve first molar

rarely affect deciduous teeth

~75% age < 30 y; ~50% < 20 y

frequent complaint of pain (61%) and swelling (70%)

X-ray: radiopaque mass that is fused to tooth root and is surrounded by a thin radiolucent rim

Cementoblastoma:– Dense masses of acellular

cementum-like material in a fibrous stroma, blend with the root of tooth

– zone of unmineralized tissue at periphery, often being arranged in radiating column

– cementum-like tissue contains many reversal lines and has a pagetoid pattern

– there are many cementoblasts, cementoclasts in the spaces between the radiating trabeculae of calcified tissue; sometimes show plump, hyperchromatic, and pleomorphic

Cementoblastoma

Treatment:– extraction of tooth together with the attached calcified mass

– prognosis is excellent

– cementoblastoma has continued growth potential; if incomplete removed, the mass will continue to grow

Clear cell odontogenic carcinomaRare jaw tumor

First described by Hansen in 1985

most cases are older than 50 years

mandible (> 80%): pain, bone expansion

aggressive lesion: invade adjacent structure, metastasize to regional LN and lungs, tendency to recur

X-ray: ill-defined or irregular radiolucency

Histo: islands or sheets of tumor epithelium, with clear cytoplasm, in a cellular fibrous stroma; the clear cells are uniform in size with a central or eccentric nucleus; clear cells contain glycogen, negative for mucin, alcian blue, oil red O


Clear Cell Odontogenic Carcinoma

Differential diagnoses:

– clear cell variant of ameloblastoma, calcifying epithelial odontogenic tumor, mucoepidermoid carcinoma

– metastatic renal cell carcinoma

Treatment:

– wide excision with prolonged follow-up

– radical surgery

– regional LN involvement 25%

Primary intraosseous carcinoma

Arise from rests of odontogenic epitheliummean age 51 y (4-76 years)posterior mandibleslight pain, paresthesia mental area, tooth mobilityill-defined radiolucencyMicroscopic features same as squamous cell carcinoma

Treatment:– Surgical resection, radiation, chemotherapy– No association between metastasis and survival time

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JAW BONE LESIONS ODONTOGENIC TUMORS