Dx: Ameloblastic Fibroma

KEY FACTS

Terminology Synonyms: Soft odontoma, mixed odontogenic tumor, fibroadamantoblastoma, granular cell ameloblastic fibroma Definition: Benign odontogenic tumor containing ectomesenchyme resembling dental papilla and epithelial strands and nests resembling dental lamina and enamel organ Imaging Well-defined, corticated, often expansile radiolucency without evidence of tooth structures or calcifications within Associated with crown of unerupted tooth (pericoronal) in many cases Usually posterior mandible of young patients Unilocular or multilocular radiolucency CBCT best demonstrates expansion and loculations Top Differential Diagnoses If pericoronal: Hyperplastic follicular space, dentigerous cyst, unicystic ameloblastoma, keratocystic odontogenic tumor (KOT) If multilocular: Ameloblastoma, central giant cell granuloma, KOT, odontogenic myxoma Clinical Issues Slow-growing but can reach large size Recurrence (~ 33%) higher with curettage, less with radical resection Malignant transformation reported at ~ 10% Radiographic follow-up recommended Diagnostic Checklist If pericoronal, look for hydraulic expansion to rule out dentigerous cyst (most common) If small, consider immature odontoma, ameloblastic fibro-odontoma, and ameloblastic odontoma
TERMINOLOGY

Abbreviations Ameloblastic fibroma (AF) Synonyms Soft odontoma Mixed odontogenic tumor Fibroadamantoblastoma Granular cell ameloblastic fibroma Definitions Benign odontogenic tumor containing ectomesenchyme resembling dental papilla and epithelial strands and nests resembling dental lamina and enamel organ
IMAGING

General Features Best diagnostic clue Well-defined, corticated, often expansile radiolucency without calcifications Associated with crown of unerupted tooth (pericoronal) in many cases Usually posterior mandible of young patients Location

Mandible > maxilla Posterior > anterior

Size: Small to very large (< 1 cm to 16 cm) Morphology Well-defined unilocular or multilocular radiolucency Typical neoplastic expansion: May form acute angles with remaining cortex Imaging Recommendations Best imaging tool CBCT will best show expansion Panoramic and occlusal views Protocol advice: Bone window Radiographic Findings Intraoral plain film Well-defined radiolucency without evidence of hard tissue structures Most often associated with crown of impacted or unerupted tooth (pericoronal) Corticated border Extraoral plain film Panoramic radiograph will demonstrate expansion in cephalad-caudal direction if present Expansion and thinning of inferior cortex of mandible and external oblique ridge Expansion into maxillary sinus or nasal cavity Loculations may be present CT Findings CBCT Demonstrates buccal-lingual expansion Loculations may be more evident Large lesions may perforate buccal and lingual cortices
DIFFERENTIAL DIAGNOSIS

Hyperplastic Follicular Space Enlarged follicular space around crown of unerupted tooth Well-defined pericoronal radiolucency Suspect pathology if pericoronal space > 3 mm Look for morphology to follow outline of tooth crown
Dentigerous Cyst

Accumulation of fluid between reduced enamel epithelium and crown of unerupted or impacted tooth Most common pericoronal radiolucency: Well-defined, unilocular Follicular space takes on more rounded appearance Hydraulic expansion: Expanded cortex meets normal cortex at equal obtuse angles May not be able to differentiate if small
Unicystic (Mural) Ameloblastoma

Ameloblastoma arising in wall of cyst (most commonly dentigerous cyst) Once infiltrates bone, behaves as ameloblastoma:May appear multilocular
Keratocystic Odontogenic Tumor (KOT)

Odontogenic neoplasm with thin para- or ortho-keratinized epithelium Does not expand significantly in mandible Can be unilocular, multilocular, or pericoronal

Ameloblastoma

Neoplasm of odontogenic epithelium Multilocular radiolucency Posterior mandible Older age group
Central Giant Cell Granuloma (CGCG)

Reactive nonodontogenic lesion Usually multilocular and expansile Predilection for anterior mandible
Never pericoronal Odontogenic Myxoma

Benign neoplasm of odontogenic ectomesenchyme Multilocular with straight septa making geometric shapes and letters
Immature Odontoma

Hamartoma of odontogenic epithelium and ectomesenchyme Produces radiopaque/high-density areas consistent with tooth structure Unilocular May be completely radiolucent prior to maturation and therefore unable to differentiate
Immature Ameloblastic Fibro-odontoma (AFO)

Mixed odontogenic neoplasm Produces less calcified tooth structure than odontoma Immature Ameloblastic Odontoma (AO) Mixed odontogenic neoplasm More aggressive than odontoma and AFO Very rare
PATHOLOGY

General Features Etiology: Unknown Genetics: Alteration of p53 gene in malignant transformations Associated abnormalities Has been reported in association with calcifying odontogenic cyst (Gorlin cyst) Strands of odontogenic epithelium are found in hypercellular connective tissue nodules located in cystic wall Gross Pathologic & Surgical Features Encapsulated tumor mass Microscopic Features Strands and nests of odontogenic epithelium in immature fibrous connective tissue stroma resembling dental papilla Contains no hard tissue (tooth) structures Odontoma, ameloblastic fibro-odontoma, and ameloblastic odontoma, which contain tooth structure, may look similar prior to maturation when calcifications may not be radiographically evident Concept that AF represents immature form of odontoma, AFO, or AO is not supported by current data Several residual and recurrent AFs have not demonstrated further maturation AF is occasionally observed in older age group beyond period of odontogenesis Malignant transformation to ameloblastic fibrosarcoma occurs in mesenchymal component: Increased cellularity, mitosis, and anaplasia

Epithelial component disappears Rarely metastasizes Malignant transformation to ameloblastic carcinosarcoma has been reported but is extremely rare Epithelial component is retained and exhibits spectrum from normal palisading ameloblasts to frankly malignant cells showing pleomorphism and hyperchromatic nuclei More likely to occur after multiple resections of AF Metastasis to lungs and regional lymph nodes
CLINICAL ISSUES

Presentation Most common signs/symptoms Hard swelling of jaws Associated with multilocular lesions Failure of eruption of involved tooth Other signs/symptoms Occasionally pain &/or drainage Symptomatic patients usually have multilocular lesions Demographics Age Childhood/adolescence Mean age ~ 15 years Gender: Slight male predilection Natural History & Prognosis Slow growing but can reach large size Recurrence (~ 33%) directly related to treatment modality Recurrence higher with more conservative treatment Longer period of nonrecurrence with more radical treatment One study found recurrence rates of 41% and 69% at 5 and 10 years, respectively Malignant transformation reported at ~ 10% Malignant transformation rate of 10% and 22% at 5 and 10 years respectively Malignant transformation less likely to occur in patients younger than 22 years May recur multiple times and even cause death Distant metastases rare Treatment Curettage Enucleation Simple excision Radical resection for larger tumors Close radiographic follow-up recommended
DIAGNOSTIC CHECKLIST

Consider Odontoma, AFO, and AO if small In early stages these lesions may be completely radiolucent Ameloblastoma, central giant cell granuloma, and keratocystic odontogenic tumor if multilocular Image Interpretation Pearls If pericoronal, look for hydraulic expansion to rule out dentigerous cyst, which is most common pericoronal radiolucency