Abstract
Aim
Purpose of this study was to review a single Institution experience and results of management of extraskeletal osteosarcoma (OGS), with emphasis on the role of combined treatment consisting of surgery and adjuvant chemotherapy.
Method
Retrospective study of 48 patients observed 1966- 2007 was undertaken: 36 patients were managed at our Institution while 12 cases were consultations and not included in this study. Clinico-pathologic features and details of treatment of all 36 patients were correlated with outcome. Updated follow-up was available in all patients.
Results
There were 21 males and 15 females, mean age was 53.6+/−19.3 years (range 14-84 yrs); 23 patients (63.9%) presented with localised disease while distant metastases were detected in 13 patients (36.1%). Primary tumour involved the extremities in 25 patients (69.4%) and the girdles or trunk in 11 (30.6%). Surgery consisted of a limb-salvage procedure in 25 cases (69.4%) and amputation in 9 (25%) while 2 patients refused surgery (5.6%). Multiagent chemotherapy was administered in 19 patients (52.8%). At mean follow-up of 5.8 years, 23 patients had died of disease, expected 5 and 10 year overall survival rates were 41% and 31%. Tumour volume (p=0.01) and age at presentation (p=0.07) were the most important predictors of survival. Disease-specific survival rate was 43.5% for patients with localised disease and 23% in patients with metastasis; difference did not reach statistical significance. Addition of adjuvant chemotherapy to local management was associated with improved disease-free survival in patients with localised disease (60% vs 31%, p=0.09).
Conclusion
Extraskeletal OGS was associated with overall worse prognosis than skeletal OGS despite multimodal management. Incidence of metastasis at diagnosis (36.1%) was higher than usually observed in skeletal OGS. Tumour volume and age were significant factors affecting the outcome while adjuvant chemotherapy showed a trend towards improved survival in patients with localised disease.
