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Osteosarcoma in fibrous dysplasia
1Department of Diagnostic Radiology, Onze Lieve Vrouwe Gasthuis, Amsterdam, The Netherlands.
Skeletal Radiology
|January 1, 1988
Summary
Osteosarcomatous transformation can occur in fibrous dysplasia of the bone. This rare complication was observed in two patients with polyostotic fibrous dysplasia, with no prior radiation exposure.
Area of Science:
- Orthopedic Oncology
- Bone Pathology
Background:
- Fibrous dysplasia is a benign bone disorder characterized by abnormal bone development.
- Malignant transformation into osteosarcoma is a rare but serious complication of fibrous dysplasia.
Observation:
- Two cases of osteosarcomatous transformation in fibrous dysplasia are presented.
- One case involved the skull, and the other involved the iliac bone.
- Both patients had a history of long-standing polyostotic fibrous dysplasia and no history of radiation therapy.
Findings:
- The incidence of sarcomatous transformation in fibrous dysplasia, based on Netherlands Committee on Bone Tumours data, is 0.5%.
- The actual incidence of spontaneous malignant transformation may be lower than suggested when strict diagnostic criteria are applied.
Implications:
- Highlights the importance of vigilance for malignant changes in patients with polyostotic fibrous dysplasia.
- Emphasizes the need for careful evaluation and strict criteria when diagnosing spontaneous sarcomatous transformation.
- Contributes to understanding the rare but significant risks associated with fibrous dysplasia.