Su David G, Brown Leanne M, Bansal Varun V, Bakkila Baylee, Concors Seth J, Turaga Kiran K, Gunderson Craig, Bergsland Emily, Strosberg Jonathan R, Halfdanarson Thorvardur R, Metz David C, Kunstman John W, Kunz Pamela L, Gangi Alexandra
Yale School of Medicine, New Haven, Connecticut, USA.
Emory University, Atlanta, Georgia, USA.
Cancer. 2025 Jul 1;131(13):e35871. doi: 10.1002/cncr.35871.
Neuroendocrine neoplasms (NENs) with peritoneal metastases (PM) represent a complex clinical challenge because of low incidence and heterogeneous phenotypes. This commentary describes the results of a national consensus aimed at addressing clinical management of patients with NENs and PM. An update of the 2018 Chicago consensus guidelines was conducted with a modified Delphi technique, which encompassed two rounds of voting. The levels of agreement for various pathway blocks were assessed, and key systemic therapy concepts were summarized by content experts. Supporting evidence was evaluated via a rapid literature review. Overall, the level of evidence for the management of PM in this disease was universally low. In total, 107 participants responded in the first round, with 88 of 107 (82%) participating in the second round. Strong consensus (>90%) was achieved in five of seven (71%) and seven of seven (100%) blocks in rounds 1 and 2, respectively. A multidisciplinary approach including psychosocial and wellness assessments received a strong positive recommendation. Management of NENs with PM was organized according to disease grade and symptom profiles. In grade 1 and 2 well-differentiated NENs, cytoreductive surgery received strong support (>95%) after the management of functional syndromes (if present). For grade 3 well-differentiated NENs, systemic therapy is the primary recommendation, with surgical resection considered in select cases. Given the limited evidence, the consensus-driven clinical pathway offers vital clinical guidance for the management of NENs with PM. The need for high-quality evidence remains critical to the field.
伴有腹膜转移（PM）的神经内分泌肿瘤（NENs）由于发病率低和表型异质性，是一个复杂的临床挑战。本评论描述了一项旨在解决NENs和PM患者临床管理问题的全国性共识的结果。采用改良德尔菲技术对2018年芝加哥共识指南进行了更新，该技术包括两轮投票。评估了各种途径模块的一致程度，内容专家总结了关键的全身治疗概念。通过快速文献综述评估了支持证据。总体而言，该疾病中PM管理的证据水平普遍较低。第一轮共有107名参与者回复，其中107名中的88名（82%）参与了第二轮。第一轮和第二轮分别在七个模块中的五个（71%）和七个（100%）模块中达成了强烈共识（>90%）。包括心理社会和健康评估在内的多学科方法获得了强烈的积极推荐。伴有PM的NENs的管理根据疾病分级和症状特征进行组织。在1级和2级高分化NENs中，在处理功能性综合征（如果存在）后，减瘤手术获得了强烈支持（>95%）。对于3级高分化NENs，全身治疗是主要推荐，在特定情况下考虑手术切除。鉴于证据有限，基于共识的临床路径为伴有PM的NENs的管理提供了重要的临床指导。高质量证据的需求对该领域仍然至关重要。
