From the Department of Surgery (Al Qahtani M), Multi Organ Transplant Center; from the Department of Surgery (Bojal), King Fahad Specialist Hospital; and from the College of Medicine (Alqarzaie, Alqahtani A) Imam Abdulrahman Bin Faisal University, Dammam, Kingdom of Saudi Arabia.
Saudi Med J. 2021 Mar;42(3):332-337. doi: 10.15537/smj.2021.42.3.20200490.
Pancreatic neuroendocrine tumors are rare with an incident rate of 5 cases per million individuals. Tuberous sclerosis complex is an autosomal dominant disease. This disease involves multisystem and occurs in one out of every 6,000-10,000 individuals. In this study, we describe a 47-year-old male known tuberous sclerosis patient with an insulinoma. The tumor was incidentally detected in follow-up imaging for a previous ampulla of Vater tubular adenoma. However, the patient reported symptoms of hypoglycemia. The insulinoma was enucleated successfully. Histopathology revealed a well-differentiated, grade one neuroendocrine tumor measuring around 2 cm in diameter. Seven cases were reported in the literature of tuberous sclerosis-associated insulinoma. The 7 reported cases had different hypoglycemia related symptoms. The reported tumors varied in size and location on the pancreas. This paper details the eighth case worldwide where an insulinoma occurred in a tuberous sclerosis patient.
胰腺神经内分泌肿瘤较为罕见，发病率为每百万个体中有 5 例。结节性硬化症是一种常染色体显性遗传疾病。这种疾病涉及多系统，每 6000-10000 个人中就有 1 个人会发病。在本研究中，我们描述了一名 47 岁的男性结节性硬化症患者合并胰岛素瘤。该肿瘤是在随访先前的壶腹管状腺瘤影像学检查时偶然发现的。然而，该患者报告有低血糖症状。成功进行了肿瘤剜除术。组织病理学显示为直径约 2 厘米的分化良好的 1 级神经内分泌肿瘤。文献中报道了 7 例与结节性硬化症相关的胰岛素瘤。这 7 例报告的病例有不同的与低血糖相关的症状。报告的肿瘤在胰腺上的大小和位置各不相同。本文详细介绍了全球第 8 例发生在结节性硬化症患者中的胰岛素瘤病例。
