Christina V. Angeles

Retroperitoneal sarcomas are extremely rare, comprising <15% of primary sarcomas. Distant metastasis occurs in about 20% of cases, with pulmonary and hepatic metastasis as the most common sites of hematogenous spread. Although surgical resection is well established as the main treatment of localized primary disease, there are limited guidelines for the surgical treatment of intra-abdominal and distant metastases. There are inadequate systemic treatment options for patients with metastatic sarcoma, thereby necessitating the consideration of surgical options in carefully selected patients. Key points to consider include tumor biology, patient fitness and co-morbidities, overall prognosis, and goals of care. Multidisciplinary sarcoma tumor board discussion for each case is an essential practice in order to deliver the best care to these patients. The purpose of this review is to summarize the published literature on the past and present role of surgery in the treatment of oligometastatic retroperitoneal sarcoma in order to inform the management of this difficult disease.