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Case report 728: Desmoplastic fibroma
J M Savy1, J D Laredo, A Prier
1Services de Rhumatologie, Hôpital Saint-Antoine, Paris, France.
Skeletal Radiology
|January 1, 1992
Summary
A rare giant cell tumor (DF) was found in a 69-year-old woman's sacrum, presenting as a lytic lesion. This tumor, typically seen in younger patients, showed very slow growth over 9 years.
Area of Science:
- Orthopedic Oncology
- Skeletal Radiology
- Tumor Pathology
Background:
- Desmoid fibromatosis (DF) is a rare neoplastic proliferation of fibroblasts, typically affecting young adults.
- Spinal and sacral locations for DF are exceptionally uncommon, posing diagnostic challenges.
- Recurrent sciatica can be an atypical presenting symptom of sacral bone lesions.
Observation:
- A 69-year-old female presented with sciatica and an expanding lytic lesion on the first sacral vertebra.
- Radiological studies over 9 years indicated minimal tumor progression, suggesting a slow growth rate.
- Histological examination confirmed the lesion as desmoid fibromatosis (DF).
Findings:
- This case highlights an unusual presentation of DF in an elderly patient's sacrum.
- The slow-growing nature of the tumor was evidenced by its stability over nearly a decade.
- Distinguishing DF from other bone tumors like low-grade fibrosarcoma or chordoma is critical.
Implications:
- This case expands the known clinical spectrum and location of desmoid fibromatosis.
- It underscores the importance of considering rare diagnoses in atypical presentations.
- Accurate differentiation of DF from malignant neoplasms is crucial for appropriate patient management.