Division of Endocrinology and Diabetes, Agamenon Magalhães Hospital, University of Pernambuco Medical School, Recife, PE, Brazil.
Department of Pathology, Federal University of Pernambuco, Recife, PE, Brazil.
Am J Case Rep. 2020 Jul 6;21:e922778. doi: 10.12659/AJCR.922778.
BACKGROUND Nesidioblastosis is a rare disease that is part of the differential diagnosis of pancreatogenic hyperinsulinemic hypoglycemia (PHH) in patients whose imaging studies do not localize insulinoma. Pancreatic heterotopia is a rare congenital abnormality characterized by pancreatic tissue anatomically separated from the main gland and found in 0.5% of abdominal surgeries. The purpose of this article is to provide a systematic review of the literature on nesidioblastosis in pancreatic ectopic tissue and to describe a case of the co-occurrence of these 2 rare conditions. CASE REPORT A 32-year-old man presented with adrenergic and neuroglycopenic symptoms, with laboratory-confirmed hyperinsulinemic hypoglycemia. There was no evidence of tumors on abdominal CT scan and MRI. Celiac trunk sampling with a calcium stimulation test was done, which showed an insulin gradient in the gastroduodenal artery. However, the intraoperative ultrasound showed a small nodule located at the pancreatic tail, leading to distal pancreatectomy. The histologic examination showed nesidioblastosis associated with pancreatic heterotopia. The patient remained asymptomatic after distal pancreatectomy. CONCLUSIONS Nesidioblastosis accounts for 0.5%-5% of all cases of PHH, with a histology showing hypertrophy and hyperplasia of pancreatic islets. Pancreatic heterotopia is a rare congenital anomaly resulting from failure of pancreatic cell migration, and is found as an incidentaloma in imaging or surgeries. Although it is a rare disease, nesidioblastosis should be considered in the investigation of hypoglycemia, even in the rare presentation of nesidioblastosis in patients with pancreatic heterotopy.
成胰岛细胞瘤病是一种罕见疾病，属于影像学检查不能定位胰岛细胞瘤的胰源性高胰岛素血症性低血糖症（PHH）的鉴别诊断之一。胰腺异位是一种罕见的先天性异常，其特征是胰腺组织在解剖上与主胰分离，在 0.5%的腹部手术中发现。本文的目的是对胰腺异位组织中的成胰岛细胞瘤病文献进行系统综述，并描述这两种罕见疾病同时发生的病例。
一名 32 岁男性出现肾上腺素能和神经低血糖症状，实验室证实为高胰岛素血症性低血糖症。腹部 CT 扫描和 MRI 均未见肿瘤证据。进行了腹腔动脉取样和钙刺激试验，结果显示胃十二指肠动脉存在胰岛素梯度。然而，术中超声显示胰腺尾部有一个小结节，导致行远端胰腺切除术。组织学检查显示与胰腺异位相关的成胰岛细胞瘤病。远端胰腺切除术后，患者无症状。
成胰岛细胞瘤病占所有 PHH 病例的 0.5%-5%，其组织学表现为胰岛细胞肥大和增生。胰腺异位是一种罕见的先天性异常，是由于胰腺细胞迁移失败导致的，在影像学或手术中偶然发现。尽管这是一种罕见疾病，但即使在胰腺异位患者中罕见表现为成胰岛细胞瘤病时，也应考虑低血糖症的病因检查。
