Dx: Desmoplastic Fibroma

KEY FACTS

Terminology Definition: Rare myofibroblastic tumor that accounts for < 1% of all bone neoplasms Imaging Multilocular radiolucency with coarse straight septa Mandible > > maxilla Posterior > anterior; especially ramus Multilocular > > unilocular (3:1) Well defined > ill defined Root divergence or resorption Expansion or cortical perforation (29% of cases) Periosteal reactions may occasionally be seen: Sunray, mimicking osteogenic sarcoma CBCT/CT: Better demonstrates locularity and any cortical erosion or periosteal reactions MR for cases with cortical perforation Determines extent of soft tissue involvement Top Differential Diagnoses Aneurysmal bone cyst Odontogenic myxoma
Ameloblastoma Central hemangioma Central odontogenic fibroma Simple (traumatic) bone cyst

Clinical Issues Painless expansile mass 1st to 5th decade; 84% < 30 years; mean = 16 years Recommended treatment: Resection or wide excision 70% recurrence withcurettage Diagnostic Checklist Location below mandibular canal rules out odontogenic lesions
TERMINOLOGY

Abbreviations Desmoplastic fibroma (DF) Synonyms Aggressive fibromatosis is soft tissue counterpart Definitions Rare myofibroblastic tumor that accounts for < 1% of all bone neoplasms
IMAGING

General Features Best diagnostic clue: Multilocular radiolucency with coarse straight septa

Location Jaws > femur > pelvic bones > radius > tibia Mandible > > maxilla Posterior > anterior; especially ramus Morphology Multilocular > > unilocular (3:1) Smaller lesions: Unilocular and may resemble simple bone cyst Larger lesions: Multilocular with coarse straight septa Well defined > ill defined Radiographic Findings Intraoral and extraoral plain film Root divergence or resorption
Expansion or cortical perforation (29% of cases)

Periosteal reactions may occasionally be seen: Sunray, mimicking osteogenic sarcoma Displacement of mandibular canal may be seen Epicenter below mandibular canal rules out odontogenic lesions CT Findings CBCT and bone CT Better demonstrates locularity and any cortical erosion or periosteal reactions Imaging Recommendations Best imaging tool CBCT/CT to examine for cortical involvement and periosteal reactions MR for cases with cortical perforation Determines extent of soft tissue involvement
DIFFERENTIAL DIAGNOSIS

Aneurysmal Bone Cyst Multilocular radiolucency with finer, wispy septa Same age group; rapidly expanding Fluid-fluid levels evident within loculations
Odontogenic Myxoma (Myxofibroma) Multilocular radiolucency with straight septa

Epicenter above mandibular canal

Ameloblastoma

Multilocular radiolucency with curved or "soap bubble" loculations Epicenter above mandibular canal; older age group Commonly posterior mandible
Central Hemangioma

Multilocular radiolucency; same age group

Central Odontogenic Fibroma Multilocular = unilocular; root resorption

Older age group; females > males

Simple (Traumatic) Bone Cyst Unilocular radiolucency with corticated border

May mimic small unilocular lesion of DF

Eosinophilic Granuloma

Unilocular ("punched-out") radiolucency without corticated border Commonly in posterior mandible

PATHOLOGY

General Features Associated abnormalities DF may appear as part of Gardner syndrome Multiple osteomas, supernumerary teeth, sebaceous cysts Reported association with tuberous sclerosis Multiple hamartomatous proliferations involving variety of organ systems; sclerotic masses in cerebrum AD inherited or sporadic mutations Microscopic Features Small elongated fibroblasts; abundant collagen fibers May appear similar to well-differentiated fibrosarcoma Some regard DFs as potentially malignant Actin positive; vimentin strongly positive Biopsy center of lesion to avoid reactive bone misdiagnosis of fibro-osseous lesion or osteosarcoma
CLINICAL ISSUES

Presentation Most common signs/symptoms: Painless expansile mass Other signs/symptoms Limited opening/trismus Pain occasionally, especially if TMJ involved Tooth mobility Exophthalmos Demographics Age: 1st to 5th decade; 84% < 30 years; mean = 16 years Gender: Males = females Natural History & Prognosis Aggressive benign tumor; morbidity may be high Long-term follow-up to monitor for recurrence Treatment Resection or wide excision Simple excision or enucleation: 20-40% recurrence Curettage: 70% recurrence Radiation therapy contraindicated because of risk of sarcoma induction
DIAGNOSTIC CHECKLIST

Image Interpretation Pearls Location below mandibular canal rules out odontogenic lesions