［摘要］ 神经内分泌肿瘤（neuroendocrine neoplasms，NENs）是一类起源于肽能神经元和神经内分泌细胞，具有神经内分泌分化特性并表达神经内分泌标志物的少见肿瘤，可发生于全身各处，以肺和胃肠胰最为常见。国内外研究均显示，NENs的发病率在不断上升。NENs的异质性较高，可起源于多个组织和器官，包括垂体、甲状腺、甲状旁腺、皮肤、支气管肺及胸腺、胃肠胰、肾上腺、生殖泌尿器官等，且同一组织或器官起源的NENs分类、分级不同时，亦有显著不同的生物学行为。NENs的高度异质性决定其诊断的困难和复杂性，除了临床症状，还需包括特殊的生物标志物、内镜、超声、计算机体层成像（computed tomography，CT）、磁共振成像（magnetic resonance imaging，MRI）等常规影像学检查以及各种功能影像学检查手段进行整合诊断。此外，NENs的治疗方式也涵盖了内镜治疗、外科治疗、介入治疗、药物治疗、放疗及肽受体放射性核素治疗（peptide receptor radionuclide therapy， PRRT）等多种手段。治疗策略的制定既要遵循指南规范，又要在多学科团队（multidisciplinary team，MDT）协作整合诊治的基础上进行个体化选择。2024年，中国抗癌协会神经内分泌肿瘤专业委员会再次组织本领域相关专家，在《中国抗癌协会神经内分泌肿瘤诊治指南（2022年版）》、其他相关国内外指南和共识、以及最新临床研究结果的基础上，制定了《中国抗癌协会神经内分泌肿瘤诊治指南（2025年版）》。本指南已在国际实践指南注册与透明化平台（Practice guideline REgistration for transPAREncy，PREPARE）上注册，注册编号为PREPARE-2024CN1158。2025年版指南对2022年版指南相应内容进行更新及修订，并进一步扩充了除胸部和胃肠胰以外的NENs，包括垂体神经内分泌瘤（pituitary neuroendocrine tumors，PitNETs）、甲状腺髓样癌（medullary thyroid carcinoma，MTC）、嗜铬细胞瘤/副神经节瘤（pheochromocytoma and paragangliomas，PPGLs）及Merkel细胞癌（Merkel cell carcinoma，MCC）的诊治推荐，以期为临床工作者提供参考。

［关键词］ 神经内分泌肿瘤；诊断；治疗；指南

［Abstract］ Neuroendocrine neoplasms (NENs) are a rare group of tumors that originated from peptidergic neurons and neuroendocrine cells, which has neuroendocrine differentiation characteristics and expresses neuroendocrine markers. NENs occur in all parts of the body, especially in lung, gastrointestinal tract and pancreas. Both domestic and foreign researches showed that the incidence of NENs is on the rise. NENs have high heterogeneity and can originate in multiple tissues and organs, including pituitary, thyroid, parathyroid, skin, bronchial lung and thymus, gastrointestinal tract and pancreas, adrenal glands, genitourinary organs, etc. At the same time, when the classification and grading of NENs originating from the same tissue or organ are different, they also have significantly different biological behaviors. The high heterogeneity of NENs determines the difficulty and complexity of its diagnosis. In addition to clinical symptoms, it also needs to include special biomarkers, endoscopy, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and various functional imaging methods for integrated diagnosis. In addition, the treatment of NENs also covers endoscopic therapy, surgical treatment, interventional therapy, drug therapy, radiotherapy and peptide receptor radionuclide therapy (PRRT). The development of treatment strategies requires not only following guidelines, but also making individual choices on the basis of multidisciplinary team (MDT) collaboration and integrated diagnosis and treatment. In 2024, the Society of Neuroendocrine Neoplasm of China Anti-Cancer Association once again organized experts in relevant fields to formulate the “China Anti-Cancer Association guideline for diagnosis and treatment of neuroendocrine neoplasms (2025 edition)” on the basis of the “China Anti-Cancer Association guideline for diagnosis and treatment of neuroendocrine neoplasm (2022 edition)”, domestic and international guidelines and consensus, as well as the latest clinical research results. The guideline had been registered on Practice guideline REgistration for transPAREncy (PREPARE) with the registration number PREPARE-2024CN1158. The 2025 edition updated and revised the relevant content from the 2022 edition and further expanded recommendations beyond thoracic, gastrointestinal tract and pancreas NENs, including pituitary neuroendocrine tumors (PitNETs), medullary thyroid carcinoma (MTC), pheochromocytoma/paragangliomas (PPGLs) and Merkel cell carcinoma (MCC), with the expectation of providing references for clinical practitioners.

［Key words］ Neuroendocrine neoplasms; Diagnosis; Treatment; Guideline