Case Report

Sarcomatous transformation in Fibrous Dysplasia- A rare case.

  Abstract :

Malignant transformation in a Fibrous Dysplasia is a rare entity. Osteosarcoma is the most common sarcoma that arises from Fibrous Dysplasia followed by fibrosarcoma and chondrosarcoma. A 70 years old gentleman presented with right knee pain for one month. He is a known case of Fibrous Dysplasia diagnosed 10 years ago. Magnetic Resonance Imaging revealed lytic lesion in distal third of right femur suggestive of Giant cell tumour. Wide resection of the distal femur was performed. Pathological examination revealed pale white, solid and fleshy nodule in the diaphysis measuring 4x3.1x2.8cm. On microscopy, a cellular spindle cell lesion was noted with cells exhibiting moderate nuclear pleomorphism, nucleomegaly, vesicular to hyperchromatic nuclei and small nucleoli. Necrosis, malignant osteoid, malignant chondroid, bizarre tumour cells or giant cells were not evident. A diagnosis of secondary Fibrosarcoma, Grade 2 arising in a Fibrous Dysplasia was rendered. We are reporting this case for its rarity in two aspects- fibrosarcoma of the bone and malignant transformation of Fibrous dysplasia. This case highlights strict scrutiny of symptoms and signs like pain, swelling or lytic bone lesions in a patient with Fibrous Dysplasia as they could be indication for a malignant transformation.

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