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关于腹膜假黏液瘤的共识和争议:对已发表的共识声明和指南的综述。

Consensuses and controversies on pseudomyxoma peritonei: a review of the published consensus statements and guidelines.

机构信息

Department of Peritoneal Cancer Surgery, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi Road, Yangfangdian Street, Haidian District, Beijing, 100038, China.

Department of Pathology, Beijing Shijitan Hospital, Capital Medical University, Beijing, 100038, China.

出版信息

Orphanet J Rare Dis. 2021 Feb 13;16(1):85. doi: 10.1186/s13023-021-01723-6.

Abstract

BACKGROUND

Pseudomyxoma peritonei (PMP) is a clinical malignant syndrome mainly originating from the appendix, with an incidence of 2-4 per million people. As a rare disease, an early and accurate diagnosis of PMP is difficult. It was not until the 1980s that the systematic study of this disease was started.

MAIN BODY

As a result of clinical and basic research progress over the last 4 decades, a comprehensive strategy based on cytoreductive surgery (CRS) + hyperthermic intraperitoneal chemotherapy (HIPEC) has been established and proved to be an effective treatment for PMP. Currently, CRS + HIPEC was recommended as the standard treatment for PMP worldwide. There are several consensuses on PMP management, playing an important role in the standardization of CRS + HIPEC. However, controversies exist among consensuses published worldwide. A systematic evaluation of PMP consensuses helps not only to standardize PMP treatment but also to identify existing controversies and point to possible solutions in the future. The controversy underlying the consensus and vice versa promotes the continuous refinement and updating of consensuses and continue to improve PMP management through a gradual and continuous process. In this traditional narrative review, we systemically evaluated the consensuses published by major national and international academic organizations, aiming to get a timely update on the treatment strategies of CRS + HIPEC on PMP.

CONCLUSION

Currently, consensuses have been reached on the following aspects: pathological classification, terminology, preoperative evaluation, eligibility for surgical treatment, maximal tumor debulking, CRS technical details, and severe adverse event classification system. However, controversies still exist regarding the HIPEC regimen, systemic chemotherapy, and early postoperative intraperitoneal chemotherapy.

摘要

背景

腹膜假性黏液瘤(PMP)是一种主要来源于阑尾的临床恶性综合征,发病率为每百万人 2-4 人。作为一种罕见疾病,PMP 的早期准确诊断较为困难。直到 20 世纪 80 年代,才开始对该疾病进行系统研究。

主要内容

经过过去 40 年的临床和基础研究进展,建立了一种基于细胞减灭术(CRS)+腹腔热灌注化疗(HIPEC)的综合策略,已被证明是治疗 PMP 的有效方法。目前,CRS+HIPEC 被推荐为全球 PMP 的标准治疗方法。针对 PMP 管理有几个共识,这些共识在 CRS+HIPEC 的规范化中发挥了重要作用。然而,全球发布的共识之间存在争议。对 PMP 共识进行系统评估不仅有助于规范 PMP 的治疗,还有助于确定现有争议,并为未来可能的解决方案指明方向。共识背后的争议反过来也推动了共识的不断完善和更新,并通过逐步和持续的过程不断改进 PMP 的管理。在本传统叙述性综述中,我们系统地评估了主要国家和国际学术组织发布的共识,旨在及时了解 CRS+HIPEC 治疗 PMP 的策略。

结论

目前,在以下方面已经达成共识:病理分类、术语、术前评估、手术治疗的适应证、最大限度的肿瘤减灭术、CRS 技术细节以及严重不良事件分类系统。然而,在 HIPEC 方案、全身化疗和术后早期腹腔内化疗方面仍存在争议。

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