Authors: T. Christopher Windham, MD; Peter W. T. Pisters, MD

Abstract

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.

Introduction

Soft tissue sarcomas are rare, with approximately 8,600 new cases diagnosed annually in the United States -- less than 1% of all newly diagnosed malignancies.[1] One third of malignant tumors that arise in the retroperitoneum are sarcomas, and approximately 15% of soft tissue sarcomas arise in the retroperitoneum.[2] Retroperitoneal sarcomas are malignant tumors arising from mesenchymal cells, which are usually located in muscle, fat, and connective tissues. Retroperitoneal sarcomas have varying clinical courses depending on their histologic subtype and grade. The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.

In this article, we discuss the pathology and prognostic features of retroperitoneal sarcomas as well as the treatment options for localized and metastatic disease.