Dx: Ameloblastoma

KEY FACTS

Terminology Definition: Benign but locally aggressive neoplasm originating from odontogenic epithelium Most common odontogenic tumor of clinical significance Imaging Well-defined expansile multilocular (80%) radiolucency in posterior mandible "Soap bubble" pattern typical; not pathognomonic Pericoronal relationship to impacted and displaced molar tooth seen in unicystic type Extensive expansion and thinning of cortices Resorption of adjacent teeth roots T2 MR: signal intensity of cystic areas T1WI C+: Solid tumor components & cyst walls show strong enhancement Mural nodule enhances in unicystic type Top Differential Diagnoses Odontogenic myxoma
Central giant cell granuloma

Aneurysmal bone cyst

Dentigerous cyst (DC) Keratocystic odontogenic tumor (KOT)

Clinical Issues 3rd-5th decade; unicystic type at younger age Slow-growing, expansile, painless mass Diagnostic Checklist Larger dentigerous cyst & KOT most difficult to differentiate from unicystic ameloblastoma Attachment at CEJ more likely indicates DC Ameloblastomas expand mandible more concentrically than KOT Follow-up for recurrence is critical
TERMINOLOGY

Synonyms Adamantinoma (old term, now describes rare low-grade malignancy of long bones), adamantoblastoma Definitions Locally aggressive, benign, odontogenic neoplasm of epithelial origin Peripheral ameloblastoma: Develops on gingiva or alveolar mucosa, not usually apparent radiographically Central ameloblastoma: Develops within jaw bones, may be uni- or multilocular radiographically Unicystic ameloblastoma: Subset of ameloblastomas; histologically presents as a single cystic entity Usually radiographically unilocular, but scalloping margins may give rise to multilocular appearance
IMAGING

General Features Best diagnostic clue: Well-defined expansile lesion with multilocular or "soap bubble" pattern in posterior mandible Location Commonly associated with impacted tooth

Mandible to maxilla ratio = 5:1

Most common site: Mandibular 3rd molar, ramus regions In maxilla: May extend into maxillary sinus or nasal fossa Peripheral ameloblastoma presents on alveolar mucosa or gingiva Size: > 2 cm at discovery in most cases Morphology Unilocular pattern: Round, expansile, cyst-like lesion Multilocular pattern (80%): Irregular or scalloped expansile shape Radiographic Findings Radiography Periphery: Well-defined, corticated border Internal structure: Reflects pathologic type Unicystic subtype: Unilocular unless scalloped periphery gives multilocular appearance Radiolucent with no internal septa Multicystic/solid subtype: Most often multilocular radiographically Bony septa are thick and curved forming round "soap bubble" loculations Desmoplastic variant: Histologic subtype of solid ameloblastoma Greater radiopaque component with sclerotic or granular bone, may mimic fibrous dysplasia Local effects Displacement and resorption of tooth roots, significant in large lesions Expansion of cortices, can be extensive Thinning and perforation of cortices Loss of anterior border of ramus with unilocular, pericoronal lesion suggestive of unicystic ameloblastoma Displacement of structures; nerve canal, floor of sinus, floor of nasal fossa CT Findings CECT Multicystic/solid lesions show strong enhancement of solid components Unicystic lesions show enhancement of wall and mural nodules Larger lesions with extraosseous extension show moderate soft tissue enhancement mixed with cystic (low density) areas Extraosseous extension is uncommon CBCT and bone CT Uni- (20%) or multilocular (80%) with scalloped borders Low-density "osteolytic" lesion "Soap bubble" pattern is typical; not pathognomonic Extensive expansion and thinning of cortices Pericoronal relationship to impacted and displaced molar tooth common Resorption of adjacent teeth, often "knife edge" MR Findings T1WI Solid tumor parts: Low to intermediate T1 signal intensity Cystic parts: Low to intermediate T1 signal intensity T2WI Solid and cystic components: High T2 signal intensity When large with extraosseous extension, high T2 signal helps differentiate from malignant tumors T1WI C+ Solid tumor components show strong enhancement Thicker enhancing walls seen than in odontogenic cysts Enhancement of "mural nodules" seen in unicystic ameloblastoma Enhancement of papillary projections or soft tissue septations Cystic areas show no enhancement Solid regions show rapid enhancement on dynamic MR, reaching maximum contrast by 60 seconds

No evidence of perineural spread Imaging Recommendations Best imaging tool: Contrast-enhanced thin section CT with soft tissue and bone algorithm Protocol advice CT best demonstrates internal morphology of septa and pattern of bone expansion Enhanced CT delineates focal enhancing mural nodules Enhanced MR imaging best defines extraosseous components & association with critical neurovascular structures Both CT & MR may be required to differentiate from other cystic lesions
DIFFERENTIAL DIAGNOSIS

Odontogenic Myxoma (Myxofibroma)

Uncommon benign tumor arising from odontogenic mesenchymal cells Multilocular lesion in posterior maxilla or mandible Finer and straighter locular septations than typical ameloblastoma Less bony expansion than ameloblastoma Greater extension between roots without resorption
Central Giant Cell Granuloma

Benign nonodontogenic reactive lesion with multilocular radiographic pattern More commonly presents anterior to 1st molars Periphery is less clearly corticated than ameloblastoma Septa are wispy and less well defined than ameloblastoma Aneurysmal Bone Cyst Children > adults Bone CT: Greatly expansile, multilocular mass Septa are more granular and wispy, like central giant cell granuloma NECT or MR: Fluid-fluid levels
Dentigerous Cyst (DC)

Unilocular cystic lesion surrounding crown of impacted tooth Lesion originating at cementoenamel junction with no remnant of follicle more likely DC CT: No enhancing mural nodule MR: Homogeneous low T1, homogeneous high T2, thin enhancing walls Unicystic ameloblastoma may mimic dentigerous cyst
Keratocystic Odontogenic Tumor (KOT)

Uni- or multilocular cystic lesion often associated with unerupted tooth Demonstrates less bony expansion in mandible with scalloping of cortices Fewer internal septa compared to multilocular/solid ameloblastoma CT: No enhancing mural nodule MR: More heterogeneous signal intensities Thinner enhancing walls Unicystic & smaller solid ameloblastoma may mimic KOT
PATHOLOGY

General Features Etiology Benign tumor arising from odontogenic epithelium (rests of enamel organ or dental lamina) Unicystic ameloblastoma from epithelial lining of odontogenic cysts; often dentigerous cyst (85%) Controversial whether unicystic ameloblastoma may arise de novo

Associated abnormalities: Unerupted 3rd molar often concurrent finding Staging, Grading, & Classification Mixed lesions such as ameloblastic fibroma, ameloblastic fibro-odontoma, and odontoameloblastoma are rare Represent a different classification of lesion Contain dental hard tissues Occur in children Gross Pathologic & Surgical Features Variable proportions of solid and cystic components Microscopic Features Proliferating sheets or islands of odontogenic epithelium in connective tissue stroma Epithelial cells are ameloblast-like Palisading, columnar cells with hyperchromatic nuclei polarized away from basement membrane Small to macroscopic cysts form in epithelial islands Histologic types: Follicular, plexiform, acanthomatous, desmoplastic, basal cell, & granular cell Unicystic type: Ameloblastic epithelium in cyst lining Epithelium may proliferate into cyst lumen (intraluminal type) or into cyst wall (mural type)
CLINICAL ISSUES

Presentation Most common signs/symptoms: Hard, painless facial or intraoral swelling Other signs/symptoms Displaced teeth Facial deformity May be no early clinical signs or symptoms Clinical profile: Adult with painless mandibular swelling Demographics Age Most commonly presents in 30-50 year olds Unicystic ameloblastoma often seen at younger age Gender: No significant gender predilection Epidemiology 2nd most common odontogenic tumor but varies by population studied Most common clinically significant odontogenic tumor 1% of odontogenic lesions Natural History & Prognosis Slow-growing, sometimes indolent, but aggressive benign neoplasm Often takes years to become symptomatic Tumor recurrence is common Recurrence rate varies by treatment but is high (15%), even with adequate treatment Unicystic tumor recurs less frequently Malignant transformation is rare (< 1%) Ameloblastic carcinoma & malignant ameloblastoma may metastasize Treatment Multilocular/solid lesions: Complete surgical resection 1 cm margin past radiologic border advocated Curettage no longer acceptable therapy Unicystic lesions without mural involvement may be treated more conservatively

Chemotherapy & radiotherapy are contraindicated Long-term follow-up required Late recurrence (> 5 years) is not uncommon
DIAGNOSTIC CHECKLIST

Consider Larger dentigerous cyst & pericoronal keratocystic odontogenic tumor most difficult to differentiate from unicystic ameloblastoma Key is pattern of bony expansion in these 2 lesions compared to ameloblastoma Image Interpretation Pearls Thick, curved septa within the lesion creating soap bubble or honeycomb pattern Look for mural nodule in unicystic lesions to differentiate from odontogenic cyst Reporting Tips Relationship to, or involvement of, inferior alveolar nerve canal in mandible Extra-alveolar extension to sublingual/submandibular space, buccal space, masticator space, maxillary sinus, pterygopalatine fissure, or orbit