Of 3000 patients diagnosed with primary malignant bone tumours and treated at our unit over the past 25 years, 234 (7.8%) were considered to be spindle cell sarcomas of bone (ie not osteosarcoma, chondrosarcoma, Ewing’s, chordoma or adamantinoma). We have analyzed their management and outcomes.

The diagnosis of these cases varied with fluctuations in the popularity of conditions such as MFH, fibrosarcoma and leiomyosarcoma with the passage of time. Treatment was with chemotherapy and surgery whenever possible. 36 patients had metastases at diagnosis and 17 had palliative treatment only because of age or infirmity. The most common site was the femur followed by the tibia, pelvis and humerus. The mean age was 45 and the mean tumour size 10.2cm at diagnosis. 25% of patients presented with a pathological fracture. Chemotherapy was used in 70% of patients the most common regime being cisplatin and doxorubicin. 35% of patients having neoadjuvant chemotherapy had a good (> 90% necrosis) response. The amputation rate was 22% and was higher in patients presenting with a fracture and in older patients not having chemotherapy.

With a mean follow up of 8 years the overall survival was 64% at 5 yrs and 58% at 10 yrs. Adverse prognostic factors included the need for amputation, older age and poor response to chemotherapy as well as a pathological fracture at presentation. The few patients with angiosarcoma fared badly but there was no difference in outcomes between patients with other diagnoses.

We conclude that patients with spindle cell sarcomas should be treated similarly to patients with osteosarcoma and can expect comparable outcomes. The histological diagnosis does not appear to predict behaviour.

Of 3000 patients diagnosed with primary malignant bone tumours and treated at our unit over the past 25 years, 234 (7.8%) were considered to be spindle cell sarcomas of bone (ie not osteosarcoma, chondrosarcoma, Ewing’s, chordoma or adamantinoma). We have analyzed their management and outcomes.

The diagnosis of these cases varied with fluctuations in the popularity of conditions such as MFH, fibrosarcoma and leiomyosarcoma with the passage of time. Treatment was with chemotherapy and surgery whenever possible. 36 patients had metastases at diagnosis and 17 had palliative treatment only because of age or infirmity. The most common site was the femur followed by the tibia, pelvis and humerus. The mean age was 45 and the mean tumour size 10.2cm at diagnosis. 25% of patients presented with a pathological fracture. Chemotherapy was used in 70% of patients the most common regime being cisplatin and doxorubicin. 35% of patients having neoadjuvant chemotherapy had a good (> 90% necrosis) response. The amputation rate was 22% and was higher in patients presenting with a fracture and in older patients not having chemotherapy.

With a mean follow up of 8 years the overall survival was 64% at 5 yrs and 58% at 10 yrs. Adverse prognostic factors included the need for amputation, older age and poor response to chemotherapy as well as a pathological fracture at presentation. The few patients with angiosarcoma fared badly but there was no difference in outcomes between patients with other diagnoses.

We conclude that patients with spindle cell sarcomas should be treated similarly to patients with osteosarcoma and can expect comparable outcomes. The histological diagnosis does not appear to predict behaviour.

Local recurrence (LR) following treatment of a sarcoma is generally accepted to be a poor prognostic sign and an indicator both of inadequate local control as well as of the failure of adjuvant treatment.

Of 2589 patients with non metastatic Ewings, osteo-sarcoma, chondrosarcoma or a soft tissue sarcoma, 316 have developed LR at some stage following initial treatment of their tumour. 120 were already known to have metastases elsewhere when they developed LR or were found to have them at time of restaging but 196 developed LR as the first sign of relapse.

The mean time to development of LR was 24 months and 72% had arisen within the first two years. Overall survival following LR was 25% at 5 years and 20% at 10 yrs. In patients with metastases at the time of LR or who were found to have them at the time of restaging the median survival was 6 months with only 12% being alive at 2 years. In those with LR as first sign of relapse median survival was 3 years with 30% long term survivors. Patients with low grade tumours had a better outcome than those with high grade – 50% being cured by further surgery. Of the high grade tumours without metastases at time of diagnosis relapsed Ewing’s had the worst prognosis with median survival of 8 months compared with 22 months for osteosarcoma, 36 months for STS and 36 months for chondrosarcoma, despite which overall survival was 16% for both Ewing’s and osteo-sarcoma patients but was 30% for chondrosarcoma and STS.

LR following sarcoma surgery is due to a combination of aggressive disease with inadequate surgery and ineffective adjuvant treatment. In isolated LR aggressive further treatment is justified with an outcome similar to that of metastatic disease.