[CCAFU's contribution to the French National Cancer Institute's reference frame: Retroperitoneal sarcomas]

Retroperitoneal sarcomas are rare tumours. The objective of this article is to propose management guidelines.

A review of the literature was performed using the PubMed search engine (1985-2013) with the key words: retroperitoneal sarcoma, prognosis, recurrence, surgery, radiation therapy, chemotherapy.

Chest, abdomen and pelvis computed tomography is the reference examination. Other examinations are optional. PET scan is not indicated for the primary diagnosis. CT-guided retroperitoneal biopsy is recommended and must be systematically performed before any management of a suspicious retroperitoneal mass. All retroperitoneal sarcomas must be registered and presented to a multidisciplinary consultation meeting devoted to the management of sarcomas (regional meetings) prior to any therapeutic intervention. Treatment is essentially surgical and is primarily designed to achieve negative surgical margins (R0). Neoadjuvant or adjuvant radiotherapy and chemotherapy can be proposed depending on the risk of progression and the resectability. The recurrence rate is related to tumour grade and surgical margins. The final prognosis is intimately related to the quality of initial management and the number of cases treated by each centre.

Retroperitoneal sarcomas have a poor prognosis. The quality of initial management directly impacts recurrence-free survival and overall survival. The prognosis is improved by multidisciplinary management conducted in a reference centre.