Objective: To update French urological guidelines on retroperitoneal sarcoma.
Methods: Comprehensive Medline search between 2016 and 2018 upon diagnosis, treatment and follow-up of retroperitoneal sarcoma. Level of evidence was evaluated.
Results: Chest, abdomen and pelvis CT is mandatory to evaluate any suspected retroperitoneal sarcoma. MRI sometimes helps surgical planning. Before histological confirmation through biopsy, the patient must be registered in the French sarcoma pathology reference network. The biopsy standard should be an extraperitoneal coaxial percutaneous sampling before any retroperitoneal mass therapeutic decision. Surgery is retroperitoneal sarcoma cornerstone. The main objective is grossly negative margins and can be technically challenging. Multimodal treatment risks and benefits must be discussed in multidisciplinary teams. The relapse rate is related to tumor grade and surgical margins.
Conclusion: Retroperitoneal sarcoma prognosis is poor and closely related to the quality of initial management. Centralization through dedicated sarcoma pathology network in a high-volume center is mandatory.
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