胰岛素瘤是产生激素的胰腺神经内分泌肿瘤 (panNEN)，估计​​发病率为每年每百万人 1-4 例。胰外胰岛素瘤极其罕见。大多数胰岛素瘤呈现惠普尔三联征：1) 症状、体征或两者均符合低血糖，2) 出现症状和体征时测得的血浆葡萄糖较低，3) 当血糖升高至正常时症状和体征缓解。非转移性胰岛素瘤现在被称为“惰性的”，而转移性胰岛素瘤被称为“侵袭性的”。据报道，惰性胰岛素瘤患者的 5 年生存率为 94-100%，而侵袭性胰岛素瘤患者的 5 年生存率为 24-67%。 5-10% 的胰岛素瘤与 1 型多发性内分泌肿瘤 (MEN1) 综合征相关。通过 CT 定位胰岛素瘤并排除或确认转移性疾病，然后对惰性局部胰岛素瘤进行 EUS 或磁共振成像 (MRI)。胰高血糖素样肽 1 受体 (GLP-1R) PET/CT 或 PET/MRI 是一种高度敏感的定位技术，适用于看似隐匿、惰性、局限性的胰岛素瘤。支持措施和生长抑素受体配体可用于控制低血糖。对于单个孤立性胰岛素瘤，治愈性手术切除仍然是首选治疗方法。在侵袭性恶性病例中，减瘤手术、生长抑素受体配体、肽受体放射性核素治疗、依维莫司、舒尼替尼和细胞毒性化疗可能是有价值的选择。© 作者 2023。由牛津大学出版社代表内分泌学会出版。

Insulinomas are hormone-producing pancreatic neuroendocrine neoplasms (panNEN) with an estimated incidence of 1-4 cases per million per year. Extrapancreatic insulinomas are extremely rare. Most insulinomas present with Whipple's triad: 1) symptoms, signs, or both consistent with hypoglycemia, 2) a low plasma glucose measured at the time of the symptoms and signs, and 3) relief of symptoms and signs when the glucose is raised to normal. Non-metastatic insulinomas are nowadays referred to as "indolent" and metastatic insulinomas as "aggressive". The 5-years-survival of patients with an indolent insulinoma has been reported to be 94-100% and for patients with an aggressive insulinoma this amounts to 24-67%. Five-10% of insulinomas are associated with the multiple endocrine neoplasia type 1 (MEN1) syndrome. Localization of the insulinoma and exclusion or confirmation of metastatic disease by CT is followed by EUS or magnetic resonance imaging (MRI) for indolent, localized insulinomas. Glucagon-like peptide 1 receptor (GLP-1R) PET/CT or PET/MRI is a highly sensitive localization technique for seemingly occult, indolent, localized insulinomas. Supportive measures and somatostatin receptor ligands can be used for the control of the hypoglycemias. For single solitary insulinomas, curative surgical excision remains the treatment of choice. In aggressive malignant cases, debulking procedures, somatostatin receptor ligands, peptide receptor radionuclide therapy, Everolimus, Sunitinib and cytotoxic chemotherapy can be valuable options.© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society.