Current diagnosis and management of retroperitoneal sarcoma.

BACKGROUND Retroperitoneal sarcomas are rare neoplasms that often present with multivisceral involvement. Treatment for these tumors requires careful decision making requiring a combination of surgery, chemotherapy, and radiation therapy. METHODS We reviewed the scientific literature pertaining to the diagnosis and management of retroperitoneal sarcomas. We also identify recent developments in treatment and discuss future trends in the care of patients with this disease. RESULTS Retroperitoneal tumors often present as large, locally advanced lesions. Evaluation of these tumors requires careful consideration of a multimodality approach. Retrospective data and historical prospective series have demonstrated the survival benefit of radical resection for these tumors with en bloc resection of involved structures. Compartmental resections in the retroperitoneum along with debulking of high-grade disease and regional therapy are controversial approaches with significant morbidity that can lead to long-term survival. The application of neoadjuvant and adjuvant therapies in select tumor histologies may improve local control and survival. CONCLUSIONS The management of retroperitoneal sarcomas requires a multidisciplinary approach and is best accomplished at high-volume centers specializing in the care of patients with these complex malignancies. Current data suggest that radical resection remains the only chance for cure and that chemotherapy and radiation therapy may confer a survival benefit.