Ewing and osteogenic sarcoma: evidence for multidisciplinary management.

STUDY DESIGN

Systematic review of the literature and consensus recommendations by an international expert focus group.

OBJECTIVE

To review and classify evidence in the literature regarding: (1) the role of neoadjuvant chemotherapy and (2) impact of extent of surgical resection on clinical outcome, particularly survival and local control, in patients with spinal Ewing sarcoma (ES) and osteosarcoma (OS).

SUMMARY OF BACKGROUND DATA

ES and OS of the spine are currently managed with multimodality treatment involving chemotherapy, radiation therapy, and surgical resection. It is currently unclear if extent of resection, for example, intralesional resection versus marginal or wide resection has an impact on survival or local control of disease.

METHODS

A systematic literature search for the years 1960 to 2008 was performed looking at publications involving treatment of spinal ES and OS. From these 208 articles, 16 were selected for analysis and were reviewed in depth. Studies were presented to a group of spinal oncology experts. Literature was graded for quality, summarized and presented to an international expert group with consensus recommendations generated.

RESULTS

For ES of the spine, 10 studies were analyzed. For OS of the spine, 6 studies were analyzed. For both ES and OS of the spine, moderate level evidence supported a strong recommendation that neoadjuvant chemotherapy offers significant improvements in local control and long-term survival and is essential in multimodality management. For spinal ES, very low level evidence supported a weak recommendation that en bloc surgical resection provides improved local control, but not improved overall survival. Radiation therapy for spinal ES may also be used for local control either alone or to supplement incomplete resection. For spinal OS, very low evidence supported a strong recommendation that en bloc resection provides improved local control and potentially improved overall survival.

CONCLUSION

Patients with ES and OS are currently managed with multiple modalities involving surgery, radiation, and chemotherapy. For both histopathologies, advances in chemotherapy have led to the greatest improvements in survival over the last few decades. Neoadjuvant therapy portents the most favorable local control and long-term survival. En bloc surgical resection may improve overall survival and decrease risk of recurrence.