Sarre Lazcano Catherine, Cardona Kenneth, Callegaro Dario
Division of Surgical Oncology, Department of Surgery, University of Toronto, Toronto, Canada.
Division of Surgical Oncology, Department of Surgery, Winship Cancer Institute, Emory University, Atlanta, GA, USA.
Ann Surg Oncol. 2025 May 9. doi: 10.1245/s10434-025-17284-y.
Retroperitoneal sarcomas (RPS) represent a rare and biologically diverse group of malignancies, distinct from soft tissue sarcomas arising in other anatomic regions. Their deep-seated location, large size at presentation and proximity to critical structures contribute to diagnostic delays, treatment challenges, and variable oncologic outcomes, particularly when managed outside high-volume referral centers. Current international guidelines emphasize the importance of preoperative biopsy, review by a sarcoma pathologist, management in a referral center, multidisciplinary case discussion, consideration for clinical trials enrollment, and prospective database inclusion as standards of care. Given that the quality of the initial surgical resection-achieving complete macroscopic resection with margins tailored to the histologic type-remains the most important modifiable prognostic factor, adherence to international best practices in surgical and multimodal treatment is crucial to optimize patient outcomes. This article reviews contemporary management strategies for adult-type RPS, emphasizing the importance of multidisciplinary expertise.
腹膜后肉瘤（RPS）是一组罕见且生物学特性多样的恶性肿瘤，与起源于其他解剖区域的软组织肉瘤不同。它们位置较深，初诊时体积较大，且靠近关键结构，这导致诊断延迟、治疗困难以及肿瘤学预后各异，尤其是在非大型转诊中心进行治疗时。当前的国际指南强调术前活检、肉瘤病理学家会诊、在转诊中心进行管理、多学科病例讨论、考虑纳入临床试验以及前瞻性数据库录入作为标准治疗方案的重要性。鉴于初次手术切除的质量——实现肉眼下完全切除并根据组织学类型确定切缘——仍然是最重要的可改变的预后因素，在手术和多模式治疗中遵循国际最佳实践对于优化患者预后至关重要。本文回顾了成人型RPS的当代管理策略，强调了多学科专业知识的重要性。
