Malignant fibrous histiocytoma of bone: the experience of the New South Wales Bone Tumour Registry.

Abstract

The New South Wales Bone Tumour Registry records for malignant fibrous histiocytoma were reviewed. Thirty-eight cases were available for analysis, representing 8% of the 506 malignant bone tumours reported to the Registry over 15 years (1977-91). The review confirmed that malignant fibrous histiocytoma is a high-grade primary bone sarcoma occurring in a wide age range in a bimodal distribution. Tumours mainly occurred around the knee or the ends of other long bones. Twenty-six per cent of cases arose in a pre-existing lesion, 4 cases post-radiotherapy, 3 cases in Paget's disease, 2 cases in bone infarcts and 1 case each in osteoblastoma, osteosarcoma and giant cell tumour. Thirty-two patients were treated by amputation or wide excision and in 11 patients this was preceded by intralesional curettage. Three patients had marginal resections and three had no surgery. Five year survival was 53% overall. Age at presentation significantly affected survival; patients under 55 years had a 5 year survival rate of 70%, compared with 19% for patients over 55 (P < 0.05). Survival was 86% at 5 years if the initial procedure was radical or wide, but only 30% if marginal or intralesional (P < 0.01). Adjuvant chemotherapy as an isolated factor did not affect survival. Rigorous adherence to the principles of surgical staging and biopsy was found to be the most successful way to treat malignant fibrous histiocytoma of bone.