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Abstract：
Retroperitoneal sarcoma (RPS) is a rare tumor classified into many histological types. It is also often detected only after it has grown to a considerable size and requires extensive resection of the surrounding organs, making it difficult to offer optimal patient-tailored management. Evidence supporting specific treatment modalities for RPS is insufficient, owing to its rarity. The Japanese clinical practice guidelines for RPS were published in December 2021, with the aim of accumulating existing evidence and indicating the optimal practice for RPS. These guidelines provide important clinical questions (CQs) concerning the diagnosis and treatment of RPS. This review, with a particular focus on primary RPS, attempts to introduce clinical problems in the diagnosis and treatment of RPS and to assess those problems along with the CQs in the guidelines. According to these guidelines, although chemotherapy and radiotherapy are expected to have therapeutic effects, the level of evidence to support these treatments is not very high at present. Accordingly, complete resection of the tumor is the first and only option for managing primary RPS. However, as with other tumors, the demand for multidisciplinary treatment for RPS is increasing. These guidelines will undoubtedly represent a milestone in clinical practice in relation to RPS in the future, and further evidence is expected to be accumulated based on the CQs that have been proposed.
摘要：
腹膜后肉瘤（RPS）是一种罕见的肿瘤，分为许多组织学类型。它也通常只有在它生长到相当大的尺寸并且需要对周围器官进行广泛切除之后才被检测到，这使得很难提供最佳的患者定制管理。支持RPS特定治疗方式的证据不足，由于它的稀有性。日本RPS临床实践指南于2021年12月发布，旨在积累现有证据并指出RPS的最佳实践。这些指南提供了有关RPS诊断和治疗的重要临床问题（CQs）。这次审查,特别关注初级RPS，尝试在RPS的诊断和治疗中引入临床问题，并评估这些问题以及指南中的CQ。根据这些准则,虽然化疗和放疗有望起到治疗作用，目前支持这些治疗的证据水平不是很高。因此，完全切除肿瘤是治疗原发性RPS的首选也是唯一的选择.然而,和其他肿瘤一样，对RPS的多学科治疗的需求正在增加。这些指南无疑将代表未来与RPS相关的临床实践的里程碑。预计将根据已提出的CQ积累进一步的证据。
