Tartaglia Andreas, Busonero Giulia, Gagliardi Lorenza, Boddi Valentina, Pieri Federica, Nizzoli Maurizio
Unit of Endocrinology and Metabolism, Morgagni Hospital, Via Carlo Forlanini 34, 47100, Forlì, Italy.
Unit of Pathology, Morgagni Hospital, Forlì, Italy.
Discov Oncol. 2022 Jul 15;13(1):66. doi: 10.1007/s12672-022-00531-z.
Hyperinsulinemic hypoglycemia is most commonly caused by a single, sporadic insulinoma. Multicentric insulinoma disease (insulinomatosis) as well as metachronous neuroendocrine tumors of the pancreas, known also as neuroendocrine adenomatosis, represent a very rare condition, if not associated with multiple endocrine neoplasia type 1 syndrome (MEN1) or Von Hippel Lindau disease. We report a 9-year follow-up of a 41-year-old woman, initially presenting with hypoglycemic syndrome caused by two insulin-producing tumors, who underwent subtotal pancreasectomy in 2012, with histology compatible with multiple small neuroendocrine tumors. An approximately 1-cm insulin-producing tumor recurred at subsequent biochemical and radiological follow-up, and was cured with the somatostatin analog octreotide as a single treatment, until remission of symptoms and complete regression of the pancreatic lesion achieved after only 16 months of treatment. The possible mechanisms for these findings are discussed and the literature is briefly reviewed.
高胰岛素血症性低血糖最常见的病因是单发的散发性胰岛素瘤。多中心胰岛素瘤病（胰岛素瘤病）以及胰腺异时性神经内分泌肿瘤，也称为神经内分泌腺瘤病，是一种非常罕见的疾病，除非与1型多发性内分泌肿瘤综合征（MEN1）或冯·希佩尔－林道病相关。我们报告了一名41岁女性的9年随访情况，该女性最初因两个胰岛素分泌肿瘤导致低血糖综合征，于2012年接受了胰腺次全切除术，组织学检查结果与多个小神经内分泌肿瘤相符。在随后的生化和影像学随访中，出现了一个约1厘米大小的胰岛素分泌肿瘤，仅用生长抑素类似物奥曲肽单一治疗就治愈了，治疗仅16个月后症状缓解，胰腺病变完全消退。本文讨论了这些发现的可能机制，并简要回顾了相关文献。
