Ann Surg Oncol. 2015 Jan;22(1):256-63. doi: 10.1245/s10434-014-3965-2. Epub 2014 Oct 15.
Retroperitoneal soft tissue sarcomas (RPS) are rare tumors that include several well-defined histologic subtypes. Although surgery is the mainstay of curative therapy, no universally accepted recommendations concerning the best management have been developed to date. Optimization of the initial approach is critical for maximizing patient outcomes.
An RPS Trans-Atlantic Working Group was established in 2013. The primary aim was to evaluate the current evidence critically and to develop a consensus document on the approach to this difficult disease. The outcome applies to primary RPS that is nonvisceral in origin. The evaluation included sarcomas of major veins (inferior vena cava, renal vein, ovarian/testicular vein), undifferentiated pleomorphic sarcoma of the psoas, and ureteric leiomyosarcoma (LMS). It excluded desmoid, lipoma and angiomyolipoma, gastrointestinal stromal tumors, visceral sarcomas such as those arising from the gut or its mesentery, uterine LMS, prostatic sarcoma, paratesticular/spermatic cord sarcoma, Ewing's sarcoma, alveolar/embryonal rhabdomyosarcoma, primitive peripheral neuro-ectodermal tumor, sarcoma arising from teratoma, carcinosarcoma, sarcomatoid carcinoma, clear cell sarcoma, radiation-induced sarcoma, paraganglioma, and malignant pheochromocytoma.
Management of RPS was evaluated from diagnosis to follow-up, and a level of evidence was attributed to each statement. This rare and complex malignancy is best managed by an experienced multidisciplinary team in a specialized referral center. The best chance of cure is at the time of primary presentation, and an individualized management plan should be made based on the statements included in this article.
International collaboration is critical for adding to the current knowledge. A prospective registry will be set up.
腹膜后软组织肉瘤（RPS）是罕见肿瘤，包括几种明确的组织学亚型。虽然手术是根治性治疗的主要手段，但迄今为止尚未制定出关于最佳治疗方案的普遍接受的建议。优化初始治疗方法对于最大化患者预后至关重要。
2013年成立了RPS跨大西洋工作组。主要目的是严格评估现有证据，并就这种疑难疾病的治疗方法制定一份共识文件。该结果适用于起源于非内脏的原发性RPS。评估包括主要静脉（下腔静脉、肾静脉、卵巢/睾丸静脉）的肉瘤、腰大肌未分化多形性肉瘤和输尿管平滑肌肉瘤（LMS）。它排除了硬纤维瘤、脂肪瘤和血管平滑肌脂肪瘤、胃肠道间质瘤、内脏肉瘤，如起源于肠道或其系膜的肉瘤、子宫LMS、前列腺肉瘤、睾丸旁/精索肉瘤、尤因肉瘤、肺泡/胚胎性横纹肌肉瘤、原始外周神经外胚层肿瘤、畸胎瘤起源的肉瘤、癌肉瘤、肉瘤样癌、透明细胞肉瘤、放射诱导的肉瘤、副神经节瘤和恶性嗜铬细胞瘤。
对RPS从诊断到随访的治疗进行了评估，并为每条陈述赋予了证据水平。这种罕见且复杂的恶性肿瘤最好由专业转诊中心经验丰富的多学科团队进行管理。治愈的最佳机会是在初次就诊时，应根据本文中的陈述制定个体化管理计划。
国际合作对于增加当前知识至关重要。将建立一个前瞻性登记系统。
