Clinical Implications of Retroperitoneal Sarcomas

Abstract

Sarcomas are uncommon malignant tumors and retroperitoneum is the less common site of origin accounting for approximately 10 % of soft tissue sarcomas. RPS present specific therapeutic challenges because of their location and frequent close association with several vital structures in the retroperitoneum. This proximity to major vessels, visceral organs, axial skeleton, and neural structures may significantly impair the ability to perform a margin-negative resection, which is the single potentially curative treatment approach in patients who have localized disease. Even in the setting of a complete resection, local recurrence is common. Because of the typically silent nature of these tumors, most RPS are large when diagnosed (>6 cm). Approximately two-thirds of cases are of high-grade histology, with liposarcomas and leiomyosarcomas representing the most common histologic findings. The initial diagnostic evaluation of patients who are suspected of having RPS should include a contrast enhanced CT of the abdomen and pelvis and MRI of the abdomen. The goal of therapy for most patients is surgical resection with negative margins. Rates of resection of visceral organs at the time of resection of RPS vary significantly by series from approximately 34–75 %. In most centers, radiotherapy is often reserved for patients who have high-grade lesions or in patients in whom a margin-positive resection is anticipated whereas the use of adjuvant chemotherapy in the management of sarcomas at any site to reduce the risk for distant disease failure is also controversial.