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阑尾肿瘤患者管理共识指南,第2部分:伴有腹膜受累的阑尾肿瘤

Consensus Guideline for the Management of Patients with Appendiceal Tumors, Part 2: Appendiceal Tumors with Peritoneal Involvement.

作者信息

Godfrey Elizabeth L, Mahoney Forest, Bansal Varun V, Su David G, Hanna David N, Lopez-Ramirez Felipe, Baron Ekaterina, Turaga Kiran K, Benson Al B, Setia Namrata, Winer Joshua H, Gunderson Craig G, Shah Rupen, Magge Deepa R, Solsky Ian, Eng Cathy, Eng Oliver S, Shergill Ardaman, Shen John Paul, Misdraji Joseph, Foote Michael B, Luo Wenyi

机构信息

Department of Surgery, Yale School of Medicine, New Haven, CT, USA.

Yale School of Medicine, New Haven, CT, USA.

出版信息

Ann Surg Oncol. 2025 Jun 25. doi: 10.1245/s10434-025-17364-z.

DOI:10.1245/s10434-025-17364-z
PMID:40560501
Abstract

BACKGROUND

Appendiceal tumors comprise a heterogeneous group of tumors that frequently disseminate to the peritoneum. Management of appendiceal tumors is lacking high quality data given their rarity and heterogeneity. In general, appendiceal tumor treatment is extrapolated in part from colorectal cancer or pooled studies, without definitive evidence of disease-specific benefit. Many practices are controversial and vary widely between institutions. A national consensus update of best management practices for appendiceal malignancies was performed to better standardize care. Herein the authors present recommendations for the management of appendiceal tumors with peritoneal involvement.

METHODS

As previously described, modified Delphi consensus was performed to update the previous 2018 Chicago consensus guideline. Recommendations were supported by using rapid systematic reviews of key issues in surgical and systemic therapy. Key pathology concepts and recommendations were synthesized in collaboration with content experts.

RESULTS

A consensus-based pathway was generated for any type of non-neuroendocrine appendiceal tumor with peritoneal involvement. The first round of Delphi consensus included 138 participants, of whom 133 (96%) participated in the second round, and greater than 90% consensus was achieved for all pathway blocks. Key items include recommending evaluation for cytoreduction to most patients with low-grade peritoneal disease who are surgical candidates, and to many patients with high-grade disease, as well as timing of systemic chemotherapy and surveillance protocols. Common pitfalls in pathologic classification and their clinical implications are also presented.

CONCLUSION

These consensus recommendations provide guidance regarding the management of appendiceal tumors with peritoneal involvement, including a review of current evidence in the management of recurrent and unresectable disease.

摘要

背景

阑尾肿瘤是一组异质性肿瘤,常扩散至腹膜。由于阑尾肿瘤罕见且具有异质性,其管理缺乏高质量数据。一般而言,阑尾肿瘤的治疗部分是从结直肠癌或汇总研究中推断而来,缺乏疾病特异性获益的确切证据。许多治疗方法存在争议,且各机构之间差异很大。为更好地规范治疗,对阑尾恶性肿瘤的最佳管理实践进行了全国性共识更新。在此,作者提出了阑尾肿瘤伴腹膜受累的管理建议。

方法

如前所述,采用改良德尔菲共识法更新了2018年芝加哥共识指南。通过对手术和全身治疗关键问题的快速系统评价来支持各项建议。关键病理学概念和建议是与内容专家合作综合得出的。

结果

针对任何类型的伴有腹膜受累的非神经内分泌性阑尾肿瘤制定了基于共识的治疗路径。第一轮德尔菲共识有138名参与者,其中133名(96%)参与了第二轮,所有治疗路径模块均达成了超过90%的共识。关键项目包括建议对大多数有手术指征的低级别腹膜疾病患者以及许多高级别疾病患者进行细胞减灭评估,以及全身化疗的时机和监测方案。还介绍了病理分类中的常见陷阱及其临床意义。

结论

这些共识建议为阑尾肿瘤伴腹膜受累的管理提供了指导,包括对复发性和不可切除疾病管理的现有证据的综述。

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本文引用的文献

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Efficacy and Safety of Atezolizumab and Bevacizumab in Appendiceal Adenocarcinoma.阿替利珠单抗联合贝伐珠单抗治疗阑尾腺癌的疗效和安全性。
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Role of colectomy in the management of appendiceal tumors: a retrospective cohort study.盲肠切除术在阑尾肿瘤治疗中的作用:一项回顾性队列研究。
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Whole-Exome Sequencing Identifies Mutation Profile and Mutation Signature-Based Clustering Associated with Prognosis in Appendiceal Pseudomyxoma Peritonei.全外显子组测序鉴定与阑尾黏液性腹膜假黏液瘤预后相关的突变谱和基于突变特征的聚类。
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Prognostic Factors in Pseudomyxoma Peritonei with Emphasis on the Predictive Role of Peritoneal Cancer Index and Tumor Markers.腹膜假黏液瘤的预后因素,重点关注腹膜癌指数和肿瘤标志物的预测作用。
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