Background: The evaluation and treatment of retroperitoneal sarcomas are challenging because the tumors are relatively rare and frequently present with advanced disease in an anatomically complex location.
Methods: We reviewed the literature on experience in the management of retroperitoneal sarcomas, and we present our own experience in the treatment of these tumors.
Results: The identification of prognostic factors other than the adequacy of resection has been inconsistent. Due to a lack of associated symptoms, retroperitoneal sarcomas smaller than 5 cm are rare. Computed tomography is the most useful tool in the evaluation of retroperitoneal tumors. Surgery, radiation therapy, and chemotherapy are treatment options, but the most important factor in the treatment of primary tumors is complete surgical resection. The role of neoadjuvant and adjuvant therapies is not defined and should be considered within the context of clinical trials.
Conclusions: Early referral of patients with retroperitoneal soft tissue tumors will help to ensure that they will receive the benefits of multidisciplinary evaluation and treatment of their disease and ready access to clinical trials.