INTRODUCTION: The purpose of this article was established by the external genitalia group CCAFU recommandations for diagnosis, treatment and monitoring of retroperitoneal sarcomas, intended for urologists. MATERIAL AND METHODS: The multidisciplinary working group has updated the 2013 guidelines, based on an exhaustive review of the literature on PubMed, valued references, level of evidence, to assign grades of recommendation. RESULTS: From a clinical suspicion evoking a RPS, computed tomography thoraco abdominal and pelvic is the gold standard. MRI is useful for surgical planning. Before the biopsy confirmation, the inclusion of the file in the French sarcoma pathology reference network should be the rule. The biopsy under scanner performed by retroperitoneal approach is recommended and should be achieve before any therapeutic management of a suspicious retroperitoneal solid mass. Treatment is primarily surgical with the main objective resection in healthy margins (R0) obtained by a technically challenging compartmental resection surgery. Instead of radiation therapy and chemotherapy within a multimodal treatment (neo adjuvant or adjuvant) is discussed based on the evolving risks and opportunities excision. The relapse rate is related to tumor grade and surgical margin. The final prognosis is closely related to the quality of initial management and the volume of cases handled by the center. CONCLUSION: The RPS has a poor prognosis. The quality of the initial management directly impacts the disease-free survival and overall survival. The multidisciplinary management coordinated within a referent care network of sarcoma pathology is an imperative necessity.
INTRODUCTION: The purpose of this article was established by the external genitalia group CCAFU recommandations for diagnosis, treatment and monitoring of retroperitoneal sarcomas, intended for urologists. MATERIAL AND METHODS: The multidisciplinary working group has updated the 2013 guidelines, based on an exhaustive review of the literature on PubMed, valued references, level of evidence, to assign grades of recommendation. RESULTS: From a clinical suspicion evoking a RPS, computed tomography thoraco abdominal and pelvic is the gold standard. MRI is useful for surgical planning. Before the biopsy confirmation, the inclusion of the file in the French sarcoma pathology reference network should be the rule. The biopsy under scanner performed by retroperitoneal approach is recommended and should be achieve before any therapeutic management of a suspicious retroperitoneal solid mass. Treatment is primarily surgical with the main objective resection in healthy margins (R0) obtained by a technically challenging compartmental resection surgery. Instead of radiation therapy and chemotherapy within a multimodal treatment (neo adjuvant or adjuvant) is discussed based on the evolving risks and opportunities excision. The relapse rate is related to tumor grade and surgical margin. The final prognosis is closely related to the quality of initial management and the volume of cases handled by the center. CONCLUSION: The RPS has a poor prognosis. The quality of the initial management directly impacts the disease-free survival and overall survival. The multidisciplinary management coordinated within a referent care network of sarcoma pathology is an imperative necessity.