Fact-sheet: Desmoplastic fibroma
Updated on 04/23/2019 at 9:56 AM
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Definition
Rare benign bone tumor (0.1 to 0.3% of bone tumors): corresponds to the osseous counterpart of soft tissue desmoid tumor.
Young adults ++, M:F 1:1
Clinical features
Locally aggressive tumor with slow progression.
May be asymptomatic, painful, or revealed by a fracture.
Distribution:
- Mandible
- Iliac bone
- Long bones (femur, tibia, humerus, radius)
Radiography
Large centered lytic metaphyseal lesion, delineated by a sclerotic rim and containing fine bony septa.
Cortical thinning or expansion may be present.
Cortical breakthrough seen in 30% of cases, potentially mimicking a malignant tumor.
Possible soft tissue extension.
MRI
Lesions usually T2 hypointense, potentially containing a few T2 hyperintense cystic areas.
Hypointense areas: hypocellular areas rich in fibrous tissue.
Heterogeneous enhancement after injection.
Modality of choice for diagnosing recurrence.
Management
Biopsy is a prerequisite prior to treatment.
Treatment: wide surgical resection.
Major complication: recurrence (72% after curettage / 17% after surgical resection)
Differential diagnosis
Giant cell tumor, simple bone cyst, Aneurysmal bone cyst, non-ossifying fibroma.
Cortical breakthrough raises suspicion for Fibrosarcoma or high-grade undifferentiated pleomorphic sarcoma.