Extraskeletal osteosarcomas: current update.

Extraskeletal osteosarcoma is a very infrequently diagnosed soft-tissue sarcoma subtype which has identical histological features to bone osteosarcoma. However, its demographics, presentation, radiology and treatment strategy differ from those of osteosarcoma. Its diagnosis can be at times challenging due to radiological and pathological mimics which have more common incidence. A multimodality approach is essential for optimizing the outcomes in extraskeletal osteosarcoma. Although there are certain caveats on inclusion of adjuvant therapies (radiotherapy and chemotherapy), in all cases surgical resection with wide local margins is considered the gold standard for adequate local control. The outcome in advanced disease remains dismal and there is a huge unmet need for prospective studies addressing the optimal treatment strategy. In this article, we review the evidence available for the management of extraskeletal osteosarcoma.