ODONTOGENIC TUMORS I.

EPITHELIAL TUMORS AMELOBLASTOMA Origin: Enamel organ Reduced enamel epithelium Odontogenic rests (Rests of Malassez and Rests of Serres) Epithelial lining of odontogenic cysts Clinical feature: 40-50 years old; no gender predilection Location is at the mandibular molar-ramus area Asymptomatic Early signs are tooth movement or malocclusion Osteolytic found in the tooth bearing areas of the jaws Unilocular (early stage) or multilocula Radiolucent (soap bubble appearance with well defined margins Clinical appearance of Oral Cancer: Leukoplakia Erythroplakia Nodular growth Swelling/enlargement Ulceration Histolopathology: All subtypes mimic enamel organ o Palisading of columnar cells around epithelial nests o Budding of tumor cells from neoplastic foci in a pattern reminiscent of tooth development Microscopic subtypes o Follicular islands of tumor cells that mimic normal dental follicle o Plexiform networking of neoplastic cells o Desmoplastic desmoplastic stroma with squamoid or elongated tumor islands o Basaloid similar to basal cell carcinoma o Granular cell central neoplastic cells exhibit prominent cytoplasmic granularity Forms: 1. Typical/Solid Ameloblastoma affects the bone; more aggressive 2. Peripheral/Extraosseous Ameloblastoma Occur in the gingiva; rarely in the buccal area 40-60 years old Origin: Overlying epithelium Rests of Serres Clinical Features: Benign Nonaggressive Does not affect the underlying bone Rare recurrence rate 3. Cystic Ameloblastoma (Unicystic Ameloblastoma) Clinical Features: Multilocular Cortical perforation in 25% of cases Histopathology: Thin, nonkeratinized epithelium Basal palisading Spongiosis Epithelial invaginations Subepithelial hyalinizations Microscopic patterns: Simple cystic Intraluminal Mural invasion

Treatment: Excision Curettage (recurrence rate of 40%) 4. Malignant Ameloblastoma Lesions that metastasize to local lymph nodes or distant organs a. Malignant Ameloblastoma Primary and metastatic lesions that are well differentiated with the characteristic histologic features of ameloblastoma b. Ameloblastic Carcinoma Lesions exhibit less microscopic differentiation More malignant because of the rapidity of formation/production 5. Primary Intraosseous Ameloblastoma Epithelial odontogenic malignancy of the mandible and maxilla arising from odontogenic rests No histologic features of ameloblastoma Primary jaw carcinoma No origin from pre-existing odontogenic cyst Affects more men seen in the mandible than the maxilla Exhibit keratin formation and half show peripheral palisading of epithelial cell nests Differentiated from acanthomatous ameloblastoma and squamous odontogenic tumor Poor prognosis with 2 year survival rate of 40% 6. Sinonasal Ameloblastoma 61 years old; male predilection Signs of nasal obstruction, epistaxis and opacification are seen Origin: totipotential sinonasal lining cells Plexiform microscopic pattern Differential Diagnosis: Calcifying epithelial odontogenic tumor and odontogenic myxoma (odontogenic tumors) Dentigerous cyst and odontogenic keratocyst (cysts) Central giant cell granuloma, ossifying fibroma, central hemangioma and idiopathic histiocytosis (nonodontogenic lesions) Treatment and Prognosis: Surgical excision solid lesions Resection/Block excision large lesions Curettage high recurrence rate CALCIFYING EPITHELIAL ODONTOGENIC TUMOR (Pindburg Tumor) Origin: Unknown cells; may be from the dental lamina remnants and stratum intermedium Clinical features: 30-50 years of age No gender predilection Mandibular molar-ramus region Peripheral lesions rarely seen in the anterior gingival Often associated with impacted teeth Unilocular or multilocular (honeycomb appearance) Radiolucent well circumscribed lesion sometimes with opaque foci a reflection of the calcified amyloid Histopathology:

Large polygonal epithelial cells in sheets/strands/nests contain nuclei that show variation in shape and size Abundant and eosinophilic cystoplasm Rare clear cell variant Extracellular amyloid of epithelial origin Liesegang rings responsible for radiopacities; concentric calcific deposits found in the amyloid material Differential diagnosis: Radiolucent o Dentigerous cyst o Odontogenic keratocyst o Ameloblastoma o Odontogenic myxoma o Benign nonodontogenic tumors Radiolucent-radiopaque o Calcified odontogenic cyst o Adenomatoid odontogenic tumor o Ameloblastic fibroodontoma o Ossifying fibroma o Osteoblastoma Treatment: Slow growing Benign No metastasis Enucleation to resection Recurrence rate (<20%) ADENOMATOID ODONTOGENIC TUMOR (Adenoameloblastoma) Clinical features: Contains ductlike or glandlike structures Rarely seen in patients over 30 years Teenagers most commonly affected Females predilection Appear in the anterior portion of the jaws of the maxilla Associated with the crowns of impacted teeth (canine) Well circumscribed unilocular lesion around the crown of an impacted tooth Radiolucent with small opaque foci reflecting the presence of calcifications Histopathology: Polyhedral to spindle cells Lobular to syncytial arrangement of cells Rosettes and ductlike structures Differential diagnosis: Dentigerous cyst (association with impacted teeth) Lateral root cyst (location) Calcifying odontogenic cyst and CEOT (opacities are evident) Treatment: Enucleation; no recurrence SQUAMOUS ODONTOGENIC TUMOR Origin: Involves the alveolar process Derived from the neoplastic transformation of the rests of Malassez Clinical features: Mandible (posterior) and maxilla (anterior) equal frequency Multicentric familial lesions 40 years old; no gender predilection Asymptomatic Signs: tenderness and tooth mobility

Semilunar well circumscribed lesion associated with the cervical region of the root Similar to ameloblastoma but lacks the columnar peripherally palisaded layer of epithelial cells Invasive and infrequently recur Differential diagnosis: Squamous cell carcinoma Acanthomatous ameloblastoma Treatment: Curettage Excision CLEAR CELL ODONTOGENIC TUMOR (Carcinoma) Origin: Unknown; probably odontogenic Clinical features: Women older than 60 years of age Mandible and maxilla Locally aggressive Occasionally painful Poorly circumscribed neoplasm composed of sheets of cells with clear cytoplasm Differential diagnosis: CEOT Central mucoepidermoid carcinoma Metastatic acinic cell carcinoma Metastatic renal cell carcinoma Ameloblastoma Hypernephroma Behaviour: Recurrence Metastasis to lungs and regional lymph nodes