Aim

To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR).

Aim

To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

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.

Aim: To investigate the outcome of our management of patients with giant cell tumour of the sacrum and draw lessons from this.

Method: Retrospective review of medical records and scans for all patients treated at our unit over the past 20 years with a giant cell tumour (GCT) of the sacrum.

Results: Of 517 patients treated at our unit for GCT over the past 20 years, only 9 (1.7%) had a GCT in the sacrum. 6 were female, 3 male with a mean age of 34 (range 15–52). All but two tumours involved the entire sacrum and there was only one purely distal to S3. The mean size was 10cm and the most common symptom was back or buttock pain. Five had abnormal neurology at diagnosis but only one presented with cauda equina syndrome. The first four patients were treated by curettage alone but two patients had intra-operative cardiac arrests and although both survived all subsequent curettages were preceeded by embolization of the feeding vessels. Of 7 patients who had curettage, 3 developed local recurrence but all were controlled with a combination of further embolisation, surgery or radiotherapy. One patient elected for treatment with radiotherapy and another had excision of the tumour distal to S3. All the patients are alive and only two patients have worse neurology than at presentation, one being impotent and one with stress incontinence. All are mobile and active at a follow up between 2 and 21 years.

Conclusion: GCT of the sacrum can be controlled with conservative surgery rather than sacrectomy. Embolisation and curettage are the preferred first option with radiotherapy as a possible adjunct. Spino-pelvic fusion may be needed if the sacrum collapses.

Aim: To identify patient, tumour or treatment factors that influence outcome in patients with radiation induced sarcoma of bone.

Method: A retrospective review of an oncology database supplemented by referral back to original records.

Results: We identified 42 patients who presented to our Unit over a 25 year period with a new sarcoma of bone following previous radiotherapy. The age of the patients at presentation ranged from 10 to 84 years of age (mean: 17 years) and the time interval from previous radiotherapy ranged from 4 to 50 years (median: 14 yrs; mean: 17 years). The median dose of radiotherapy given had been 50 Gy but there was no correlation of radiation dose with time to development of sarcoma. The pelvis was the most common site for development of sarcoma (14 cases) but breast cancer was the most common primary tumour (8 cases). 9 of the patients had metastases at the time of diagnosis of the sarcoma. Osteosacoma was the most common diagnosis (30). Treatment was by surgery and chemotherapy when indicated and 30 of the patients had treatment with curative intent. The survival rate was 41% at 5 years for those treated with curative intent but in those treated palliatively median survival was only 6 months and all had died by one year. The only factor found to be significant for survival was the ability to completely resect the tumour, thus limb sarcomas had a better prognosis (66% survival at 5 years) than central ones (12%)(p=0.009).

Conclusion: Radiation induced sarcoma is a rare complication of radiotherapy. Both surgical and oncological treatment is likely to be compromised by previous treatment the patient has received. Despite this 40% of patients will survive more than 5 years with aggressive modern treatment.

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.

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.