Retroperitoneal sarcomas.

Abstract

Retroperitoneal sarcomas are rare malignant tumors, accounting for approximately 10-20% of all soft tissue sarcomas. Because of their anatomic location and indolent nature, these tumors are usually quite extensive when first detected with little hope of improvement in the early detection rate. Improved survival rates will therefore depend on obtaining better local control through an aggressive surgical approach, making every effort to achieve a complete resection with histologic negative margins, which in most cases will require the resection of adjacent organs or vascular structures. Even with such aggressive surgical approach, continued problems with local recurrence are reported and it is important to follow patients who had undergone surgical resection very carefully in order to identify those who would benefit from reresection. The results of postoperative adjuvant irradiation and/or chemotherapy have so far been discouraging. Until there is a national registry and a national intergroup study to develop strategies and innovative trials for the effective treatment of these tumors, it is unlikely that there will be much improvement in the current survival rates.