日本《腹膜后肉瘤诊疗指南》解读与临床进展

Interpretation of the Japanese Clinical practice guidelines for the management of retroperitoneal sarcoma and clinical advances

摘要:

腹膜后肉瘤是一种发病率较低但恶性程度较高的软组织肿瘤，其诊断与治疗一直是临床研究的热点。日本肉瘤治疗研究学会等多个医学组织于2021年12月发布了《腹膜后肉瘤诊疗指南》，并于2023年4月进行了修订。该指南围绕腹膜后肿瘤的诊断、原发性腹膜后肉瘤的治疗及复发性/不可切除性腹膜后肉瘤的治疗三个方面以及由此衍生的11个临床问题给出推荐，并首次系统性提出了这一疾病的诊疗流程图，对国内腹膜后肉瘤的规范化诊疗具有重要的借鉴意义。诊断流程涵盖临床特征与影像学检查、病理诊断与活检；在治疗方面，尽管手术切除难度较大，但仍是主要的治疗手段，指南尤其强调了R₀切除。此外，化疗、放疗、粒子线治疗及靶向治疗在腹膜后肉瘤的治疗中也起到了重要作用。笔者结合临床证据与国内外现有其他指南，对该指南中有关影像与病理诊断、手术切除方面的推荐展开重点分析与讨论，并基于粒子治疗、免疫治疗等领域最新研究成果探讨了当前非手术治疗方案的有效性，展望了通过个体化治疗、联合治疗及多学科团队合作提高患者生存质量的前景。

Abstract:

Retroperitoneal sarcoma is a rare but highly malignant type of soft tissue tumor, and its diagnosis and treatment have long been focal points in clinical research. In December 2021, the Japanese Society for Sarcoma Research, together with several other medical organizations, published the Clinical practice guidelines for the management of retroperitoneal sarcoma, which were revised in April 2023. The guidelines provide recommendations on three key aspects: the diagnosis of retroperitoneal tumors, treatment of primary retroperitoneal sarcomas, and management of recurrent or unresectable cases. They also address 11 clinical questions derived from these topics and, for the first time, present a systematic diagnostic and treatment algorithm for this disease—offering important reference value for standardizing the management of retroperitoneal sarcoma in China. The diagnostic process includes assessment of clinical features, imaging evaluation, pathological diagnosis, and biopsy. Despite the technical challenges, surgical resection remains the mainstay of treatment, with a particular emphasis on achieving R₀ resection. In addition, chemotherapy, radiotherapy, particle therapy, and targeted therapy also play crucial roles. This article focuses on analyzing and discussing the guideline's recommendations on imaging, pathological diagnosis, and surgical resection, in comparison with other domestic and international guidelines. It further explores the effectiveness of current non-surgical treatment strategies based on recent advances in particle and immunotherapy, and looks ahead to the prospects of improving patient outcomes through personalized treatment, multimodal therapy, and multidisciplinary collaboration.