Bordi C, De Vita O, Pilato F P, Carfagna G, D'Adda T, Missale G, Peracchia A
Am J Clin Pathol. 1987 Aug;88(2):153-61. doi: 10.1093/ajcp/88.2.153.
Two cases of multiple islet cell tumors mostly composed of glucagon-producing cells and associated with severe ulcer disease are presented. Multiple endocrine neoplasia type I (MEN-I) was present in both patients, although symptomatically latent in case 2. Immunohistochemistry showed that glucagon (A) cells were a major cell population (i.e., accounting for at least 30% of the tumor cell population) in 24 of 43 tumors (either macroadenomas or microadenomas) studied in case 1 and in 12 of 17 tumors studied in case 2. A major pancreatic polypeptide (PP) cell population was found in 12 and 7 tumors of case 1 and 2, respectively. In contrast, insulin (B) and somatostatin (D) cells were scarce in most adenomas. Gastrin-producing cells were not identified in any tumors, despite the use of different antigastrin antisera. Extrapancreatic or residual gastrinomas were not found at postmortem examination in case 1 or on appropriate surgical inspection done 24 years after the onset of the ulcer disease in patient 2. On the basis of these and of 17 additional cases collected in the literature, it is concluded that multiple A-cell tumors of the pancreas are an expression of the MEN-I and are mostly associated with ulcer disease and/or with hypergastrinemia of frequent uncertain origin. The mechanisms regulating the nonrandom phenotypic hormonal differentiation of these genetically determined tumors remain unknown.
本文报告了两例以产生胰高血糖素的细胞为主的多发性胰岛细胞瘤，并伴有严重溃疡病。两名患者均患有I型多发性内分泌腺瘤病（MEN-I），尽管病例2在症状上处于潜伏状态。免疫组织化学显示，在病例1研究的43个肿瘤（大腺瘤或微腺瘤）中的24个以及病例2研究的17个肿瘤中的12个中，胰高血糖素（A）细胞是主要细胞群（即占肿瘤细胞群的至少30%）。在病例1和病例2的肿瘤中，分别有12个和7个发现了主要的胰多肽（PP）细胞群。相比之下，大多数腺瘤中胰岛素（B）细胞和生长抑素（D）细胞很少。尽管使用了不同的抗胃泌素抗血清，但在任何肿瘤中均未鉴定出产生胃泌素的细胞。在病例1的尸检中或在患者2溃疡病发病24年后进行的适当手术检查中均未发现胰腺外或残留的胃泌素瘤。根据这些以及从文献中收集的另外17个病例，得出结论：胰腺多发性A细胞肿瘤是MEN-I的一种表现，并且大多与溃疡病和/或来源常常不确定的高胃泌素血症相关。调节这些基因决定的肿瘤的非随机表型激素分化的机制仍然未知。
