The Diagnosis and Management of Retroperitoneal Sarcoma.

Retroperitoneal sarcoma (RPS) is a rare tumor whose diagnosis and management can be challenging and for which management requires a multidisciplinary team in a specialized center. An important part of the diagnosis-identification of the histologic subtype-depends on pathology; identifying the histologic subtype is important because this can affect prognosis and treatment options. Complete surgical resection with negative margins remains the cornerstone of treatment of nonmetastatic RPS and is the only chance for cure. In order to achieve negative margins, multivisceral en bloc resection is often necessary. Neoadjuvant therapies (chemotherapy, external beam radiation, or combination radiation and chemotherapy) are safe in well-selected patients and may be considered after careful review by a multidisciplinary sarcoma tumor board when the recurrence risk is high.