Odontogenic tumors Markéta Hermanová Odontogenic tumors nTumors derived from the dental formative tissues n nUncommon, some are exceedingly rare, represent 1 % of all tumors of oral cavity. n nDerived from epithelial, ectomesenchymal and mesenchymal tissues. n n Classification of odontogenic tumors nAccording biological behavior §Benign §Malignant n nAccording localisation §Intraosseous §Extraosseous n Classification of odontogenic tumors nHistogenetic/histomorphological classification §Epithelial -Without odontogenic mesenchyme -With odontogenic mesenchyme - §Mesenchymal § §Tumors of debatable origin -Melanotic neuroectodermal tumor of infancy -Congenital gingival granular cell tumor (epulis congenita) n n Benign epithelial odontogenic tumor §Without odontogenic mesenchyme nAmeloblastoma nSquamous odontogenic tumor nCalcifying epithelial odontogenic tumor nAdenomatoid odontogenic tumor nKeratinising cystic odontogenic tumor n §With odontogenic mesenchyme nAmeloblastic fibroma and fibrodentinoma nAmeloblastic fibro-odontoma nOdontoameloblastoma nCalcifying odontogenic cyst and dentinogenic ghost cell tumor nComplex odontoma nCompound odontoma Benign mesenchymal odontogenic tumours nOdontogenic fibroma -benign, rare, mandible, maxilla n nMyxoma -benign, relapsing, mandible, maxilla n nCementoblastoma -benign, relapsing, associated with the root of a tooth -Calcified cementum-like tissue containingscattered cells lying in lacunae Malignant odontogenic tumors nOdontogenic carcinomas nMalignant ameloblastoma/ameloblastic carcinoma nPrimary intraosseous carcinoma (without accociation with oral mucosa) nClear cell odonogenic carcinoma nMalignant variants of other epithelial tumors nMalignant change in odontogenic cysts n(metastatic spreading into lymph nodes, lungs, bones,…) n nOdontogenic sarcomas nAmeloblastic fibrosarcoma nAmeloblastic fibro-odontosarcoma Ameloblastoma nBenign, local invasive, rellapse after years n nSubtypes: -Solid/multicystic -Extraosseous/peripheral (soft tissues above mandibula, older patients) -Desmoplastic (anterior maxilla and mandible) -Unicystic n Solid multicystic ameloblastoma n n n n3.-5. decade, molar region/ascending ramus of the mandible n (70 %) + posterior maxilla nTwo main histological patterns: follicular and plexiform nVariants: basaloid, granular, acantomathous, keratoacantomathous nColumnar/cuboidal peripheral cells considered to be preameloblasts) nMature fibrous stroma, does not contain enamel or dentin Treatment: surgical resection with a margin of normal bone Unicystic ameloblastoma nYounger patients n nOften associated with an unerupted tooth, macroscopically looks like dentigerous cyst nCystic lesion lined by ameloblastomatous epithelium, with palisading of basal layer of the cells nLuminal amd mural variants nLuminal variants treated by n enucleation and curettage; n mural variants treated by resection Other benign epithelial odontogenic tumors nSquamous odontogenic tumor -benign, locally aggressive, rare, in mandible, M˃F, 4th decade -nests of squamous epithelium in fibrous stroma - nCalcifying epithelial odontogenic tumor (Pindborg´s tumor) -benign, locally aggressive, rare, in mandible, M˃F, 4th decade (molar and premolar region) -sheets of pleomorphic epithelial cells, amyloid-like material, calcification -assoc. with Gorlin-Goltz syndroma n Other benign epithelial odontogenic tumors - nAdenomatoid odontogenic tumor -benign, hamartoma?, 2nd, 3rd decade, anterior part of the maxila, often assoc. with unerupted tooth -solid nodules and tubular structures + eosinophilic material, calcification; treated by enucleation - nKeratinising cystic odontogenic tumor = odontogenic keratocyst (WHO 2005 true tumor) -benign, locally aggressive -Mandibular angle (50 %), 60 % relapsing; treated by enucleation? n Odontogenic keratocysts nBimodal age distribution – 2nd-3rd decades and 5th decade n nFew symptoms; cause little expansion; may reach large size n nUnilocular/multilocular radiolucency; may mimic dentigerous cyst n nMore common in mandibula than in maxila n nTendency to recur n nMay be multiple; associated with nevoid basal cell carcinoma syndrome Naevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome) nAD n nMultiple naevoid BCC + multiple odontogenic keratocysts + skeletal abnormalities (rib abnormalitites, vertebral deformities, polydactyly, cleft lip/palate) + calcified falx cerebri + brain tumours n nMutation in tumor supperssor gene PTCH (9q) n nMutations of PTCH affect the normal function of Hedgehog signalling pathway n nHedgehog signalling pathway controls transcription of the genes involved in the developlment, patterning, and growth of numerous tissues and organs n n Odontogenic keratocysts nThin, easily torn wall n nLined by an even layer of parakeratinized squamous epithelium n nPalisaded basal cell layer n nSatellite cysts in capsule n nTendency to recur due to difficulty of surgical removal -thin, easily ruptured wall -Projection into cancellous spaces easily torn -Satellite cysts in capsule - nCyst enlargement involves -Focal areas of active growth of the cyst wall -Extension of proliferating areas along cancellous spaces -Production of bone resorbing factors Odontogenic keratocyst odontogenní cysta Ameloblastic fibroma, fibrodentinoma, fibro-odontoma nBenign tumors, fibro-odontoma v.s. hamartoma nWell circumscribed, mainly in 1st and 2nd decade, posterior mandible nOdontogenic epithelium + cellular mesenchymal tissue nFibrodentinoma contains dentin, fibro-odontoma enamel and dentin nMust be distinguished from ameloblastoma, different treatment (curretage)! n n Calcifying cystic odontogenic tumor = calcifying odontogenic cyst (Gorlin´s cyst) nWHO 2005 true tumor nWide age range nAnterior mandible, maxilla, gingiva nCyst lined by ameloblastic epithelium, „ghost cells“→calcification, „dentinoid“ at basal layer of epithelium in supporting fibrous tissue nTend to recur, sometimes associated n with ameloblastoma nTreatment: enucleation Odontomas (true tumors?, hamartomas, congenital anomalies?) nInvaginated (dens-in-dente, dens invaginatus) nEvaginated (dens evaginatus) nEnamel pearls/enamelomas n„Double tooth“/geminated odontoma n n n nComplex odontoma nCompound odontoma Complex odontoma nDevelompmental lesion resulting in disorganized mass of dental tissue n n2nd/3rd decade, predominantly molar region mandible, omay overlie/replace a tooth n Compound odontoma n1st/2nd decade; predominantly anterior maxilla n nDevelopmental lesion resulting in the formation of a bag of discrete denticles n nDenticles comprise enamel, dentine, cementum, and pulp in their normal anatomical relationship n nSeparate dinticles identifiable on radiograph Melanotic neuroectodermal tumor of infancy Synonyms: melanotic progonoma, retinal anlage tumor,… nVery rare nTumor of infancy, 80 %<6 month, 95 % <1 year of age nF:M: 2:1 nmaxilla>mandible>skull nRapidly growing pigmented mass nLocal recurrences, metastatic (7 %): lymph nodes, liver, bones Congenital epulis of the newborns – congenital gingival granular cell tumor: n §Incisor region of the maxilla, F>M § §Closely packed granular cells covered by flattened squamous epithelium § §Benign lesion, unknown etiology n(reactive?,….neoplastic???, but unrelated to granular cell tumor of the tongue ) n n Congenital epulis of the newborns – congenital gingival granular cell tumor congenital_epulis_10_030601 1048885-1079023-2232 Take home message nOdontogenic tumors are rare, but occur n nNOT ONLY ameloblastoma n nOften benign x locally aggressive n nNot to rely only on radiography, histopathological examination of diagnosis is necessary! n nSecondary inflammation can cover some signs Thank you for your attention……..