Spindle cell sarcoma, possible leiomyosarcoma.
Surgery was performed with radical, wide resection of her distal femur and reconstruction of the knee joint and femur with segmental endoprosthesis. She did not undergo any radiation or chemotherapy.
Histologic examination of the resected tumor showed fascicular pattern of growth with low grade spindle cells and evidence of pleomorphism. Smooth muscle markers were negative. Her cancer was categorized as a grade 2 sarcoma, consistent with malignant fibrous histiocytoma according to the WHO classification of bony tumors. The patient is recovering from surgery and doing well. All of her soft tissue and bone margins were clear and there was no evidence of lymphatic or vascular invasion. The patient has a good chance for near full recovery. However, recurrence is not uncommon and she will require regular follow-up for surveillance.
Be wary of the rare bone neoplasm. Pain is the most common symptom. Most tumors are visible on x-rays, with a "ragged" or "holey" appearance. Early detection and referral are key.
Malignant fibrous histiocytoma is rare, comprising < 2% of all primary malignant bone tumors. It has a 50-60% survival rate at 5 years, with an increased survival rate in those under the age of 40. The appendicular skeleton is involved in 75% of cases.
Randall R, Albritton K, Ferney B, Layfield L. Malignant fibrous histiocytoma of soft tissue: an abandoned diagnosis. Am J Orthop 2004 Dec;33(12)602-8.
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