Gould V E, Chejfec G, Shah K, Paloyan E, Lawrence A M
Department of Pathology, Rush Medical College, Chicago, IL 60612.
Semin Diagn Pathol. 1984 Feb;1(1):43-53.
Subtotal pancreatectomy specimens from two adults with hyperinsulinemic hypoglycemia and one adult with watery diarrhea syndrome were investigated. All three specimens were originally diagnosed as "nesidioblastosis"; none had a neoplasm, and all patients were cured of their endocrine dysfunction by the surgical procedure. Tissue samples were studied by light microscopy and light microscopic immunohistochemistry for serotonin, ACTH, bombesin, calcitonin, gastrin, glucagon, insulin, HPP, somatostatin, and VIP. The results were compared with those obtained from the parallel study of ten adult pancreata obtained at autopsy from patients without pancreatic disease or endocrine dysfunction. The total endocrine cell mass was not notably greater in the pancreata from patients with endocrine dysfunction than in the controls. Both groups had an estimated 95% of the endocrine cell population organized in islets while the remaining 5% was irregularly dispersed amidst the exocrine ducts and acini. Findings more conspicuous in patients with endocrine dysfunction but not absent in the controls included: large islets, islets with irregular contours and ragged edges, and large individual islet cells with abundant cytoplasm and bizarre nuclei. Immunoreactivity for insulin, glucagon, and somatostatin was demonstrated in all cases; the ratio among these hormones in the patients with endocrine dysfunction and in the controls was similar. In the patients with hyperinsulinemic hypoglycemia, step sections sequentially stained for insulin and somatostatin showed a close topographic relationship between these cell types. In the patient with the watery diarrhea syndrome, VIP immunoreactive cells were easily identified admixed with exocrine components; they were only rarely seen within islets. We suggest that the hyperinsulinemic hypoglycemia in these adults was not due to simple quantitative abnormalities in total endocrine cell mass nor to its maldistribution nor to lack of topographic proximity between insulin and somatostatin cells. We speculate that the syndrome may be based on still unknown derangements of insulin secretion, release, and/or its degradation. In the case of the watery diarrhea syndrome, the readily identifiable VIP immunoreactive cells represent the emergence of a functional expression regarded as ectopic for the endocrine cells of the pancreas. We conclude that nesidioblastosis per se cannot be viewed as the structural basis of these endocrine dysfunctions since many of its features were present in control pancreata lacking any such association.(ABSTRACT TRUNCATED AT 400 WORDS)
对两名患有高胰岛素性低血糖症的成年人以及一名患有水样腹泻综合征的成年人的胰腺次全切除术标本进行了研究。所有三个标本最初均被诊断为“胰岛细胞增殖症”；均无肿瘤，且所有患者均通过手术治愈了内分泌功能障碍。通过光学显微镜和针对血清素、促肾上腺皮质激素、蛙皮素、降钙素、胃泌素、胰高血糖素、胰岛素、人胰多肽、生长抑素和血管活性肠肽的光学显微镜免疫组织化学对组织样本进行了研究。将结果与从对十例无胰腺疾病或内分泌功能障碍的成年人尸检获得的胰腺进行的平行研究中得到的结果进行了比较。内分泌功能障碍患者的胰腺中总内分泌细胞量并不比对照组明显更多。两组估计均有95%的内分泌细胞群组织成胰岛，而其余5%不规则地散布在外分泌导管和腺泡之间。在内分泌功能障碍患者中更明显但在对照组中并非不存在的发现包括：大胰岛、轮廓不规则且边缘参差不齐的胰岛以及具有丰富细胞质和怪异细胞核的大单个胰岛细胞。在所有病例中均显示出对胰岛素、胰高血糖素和生长抑素的免疫反应性；内分泌功能障碍患者与对照组中这些激素之间的比例相似。在患有高胰岛素性低血糖症的患者中，依次对胰岛素和生长抑素进行染色的连续切片显示了这些细胞类型之间密切的地形关系。在患有水样腹泻综合征的患者中，血管活性肠肽免疫反应性细胞很容易被识别为与外分泌成分混合；它们在胰岛内很少见。我们认为，这些成年人的高胰岛素性低血糖症并非由于总内分泌细胞量的简单定量异常、其分布不均或胰岛素和生长抑素细胞之间缺乏地形上的接近性。我们推测该综合征可能基于胰岛素分泌、释放和/或其降解的仍未知的紊乱。就水样腹泻综合征而言，易于识别的血管活性肠肽免疫反应性细胞代表了一种被视为胰腺内分泌细胞异位的功能性表达的出现。我们得出结论，胰岛细胞增殖症本身不能被视为这些内分泌功能障碍的结构基础，因为其许多特征在缺乏任何此类关联的对照胰腺中也存在。（摘要截短至400字）
