PRIMARY SPINDLE CELL SARCOMA OF BONE – AN ASSESSMENT OF OUTCOME

Abstract

To assess whether primary spindle cell sarcomas of bone behave like other primary bone sarcomas.

185 patients with primary spindle cell sarcomas of bone, that is non-osteosarcoma, non-chondrosarcoma and non-Ewings sarcoma of bone, were identified from the patient database of the Orthopaedic Oncology Service in Birmingham, UK. This database contains information on over 10, 000 patients treated in Birmingham since 1970 and collected prospectively since 1986.

Spindle cell sarcomas of bone are primary bone tumours which share the histological spindle shaped cells. They are a heterogeneous group including the cytological diagnoses of malignant fibrous histiocytoma (MFH), spindle cell sarcoma, leiomyosarcoma, fibrosarcoma, angiosarcoma and secondary sarcoma.

119 (64%) of the patients were male. The mean age was 47 years with 116 (63%) older than 40 years. Only 6 patients were low grade and 34 had metastases at presentation. 12 patients developed tumours secondarily to other pathology such as Pagets or radiotherapy. The patient’s limb was affected in 155 (84%) of cases with the distal femur being the most common site.

Treatment was with chemotherapy and surgery where indicated. Chemotherapy was with adriamycin and cis-platin most commonly and was neoadjuvant in 75% of those who had it. Of the 150 patients who had surgery, 113 (75%) had limb salvage.

Overall five year survival was 52 %. Survival in patients who had high grade limb tumours, with no metastases at presentation and which weren’t secondary tumours had a five year survival of 60 %. These results are comparable to previous studies looking at similar tumour groups. Good prognostic factors included treatment since 1980, good response (> 90% necrosis) to neo-adjuvant chemotherapy, limb tumours, age < 40 and local control. 73% of patients with local recurrence died with a mean survival of 11 months. There was no difference in outcome or behaviour between the different diagnoses although prognosis was slightly better than those of aged matched patients with osteosarcoma.

Spindle cell sarcomas of bone respond in a similar way to, if not better than, osteosarcoma when treated in the same way with chemotherapy and surgery. Consequently this should be the preferred method of management.