PDF(Pubmed)
Abstract：
The best-known etiologies of hyperinsulinemic hypoglycemia are insulinoma, non-insulinoma pancreatogenous hypoglycemic syndrome, autoimmune processes, and factitious hypoglycemia. In 2009, a disease not associated with classic genetic syndromes and characterized by the presence of multiple pancreatic lesions was described and named insulinomatosis. We present the clinical and pathologic features of four patients with the diagnosis of insulinomatosis, aggregated new clinical data, reviewed extensively the literature, and illustrated the nature and evolution of this recently recognized disease. One of our patients had isolated (without fasting hypoglycemia) postprandial hypoglycemia, an occurrence not previously reported in the literature. Furthermore, we reported the second case presenting malignant disease. All of them had persistent/recurrent hypoglycemia after the first surgery even with pathology confirming the presence of a positive insulin neuroendocrine tumor. In the literature review, 27 sporadic insulinomatosis cases were compiled. All of them had episodes of fasting hypoglycemia except one of our patients. Only two patients had malignant disease, and one of them was from our series. The suspicion of insulinomatosis can be raised before surgery in patients without genetic syndromes, with multiple tumors in the topographic investigation and in those who had persistent or recurrent hypoglycemia after surgical removal of one or more tumors. The definitive diagnosis is established by histology and immunohistochemistry and requires examination of the \"macroscopically normal pancreas.\" Our case series reinforces the marked predominance in women, the high frequency of recurrent hypoglycemia, and consequently, a definitive poor response to the usual surgical treatment.
摘要：
高胰岛素血症性低血糖最著名的病因是胰岛素瘤，非胰岛素瘤胰源性低血糖综合征，自身免疫过程，和人为的低血糖。2009年，描述了一种与经典遗传综合征无关且以存在多个胰腺病变为特征的疾病，并将其命名为胰岛素瘤病。我们介绍了4例诊断为胰岛素瘤的患者的临床和病理特征，汇总的新临床数据，广泛回顾了文献，并说明了这种最近认识到的疾病的性质和演变。我们的一名患者有孤立的（无空腹低血糖）餐后低血糖，以前文献中没有报道过的事件。此外，我们报告了第二例表现为恶性疾病的病例。即使病理证实存在阳性胰岛素神经内分泌肿瘤，他们在第一次手术后都有持续/复发的低血糖。在文献综述中,收集了27例散发性胰岛素瘤病病例。除我们的一名患者外，所有患者均有空腹低血糖发作。只有两名患者患有恶性疾病，其中一个来自我们的系列。在没有遗传综合征的患者手术前可以提高胰岛素瘤病的怀疑，在地形图研究中患有多个肿瘤，以及在手术切除一个或多个肿瘤后患有持续性或复发性低血糖的患者。明确的诊断是通过组织学和免疫组织化学建立的，需要检查宏观上正常的胰腺。“我们的案例系列加强了女性的明显优势，反复低血糖的高频率，因此，对常规手术治疗的明显反应不佳。
