Takahashi H, Nakano K, Adachi Y, Aoki N, Hajiro K, Yamamoto T, Higashizawa T, Chikugo T, Suzuki T
Second Department of Internal Medicine, Kinki University School of Medicine, Osakasayama, Japan.
Acta Pathol Jpn. 1988 May;38(5):667-82. doi: 10.1111/j.1440-1827.1988.tb02339.x.
A case of multiple nonfunctional pancreatic islet cell tumor in multiple endocrine neoplasia type I (MEN I) is reported. The patient was a 41-year-old woman who had a past history of thyroid cancer (papillary carcinoma) and hyperparathyroidism due to parathyroid adenoma. Later, a nonfunctional pituitary tumor and five nonfunctional pancreatic tumors were found simultaneously and the patient was finally diagnosed as having MEN I. Following surgical enucleation, the pancreatic tumors were histopathologically diagnosed as benign islet cell tumors. One of them (tumor 3) exhibited a solid nodular pattern while the others showed gyriform patterns. They were divided histochemically and immunohistochemically into three types: two (tumors 1 and 2) produced a single hormone (glucagon), one (tumor 3) produced five (insulin, glucagon, somatostatin, gastrin and pancreatic polypeptide) and the remaining two (tumors 4 and 5) produced two (glucagon and pancreatic polypeptide). Electron microscopically, three types of endosecretory granules were found in the tumor cells of tumor 3 but only one type was found in tumor 4. However, in the tumor 4 extract, glucagon, pancreatic polypeptide, C-peptide, somatostatin, vasoactive intestinal peptide and growth hormone releasing factor were detected by radioimmunoassay. These findings suggest that these pancreatic tumors were both multicellular and multihormonal.
报告了1例1型多发性内分泌腺瘤病（MEN I）中的多发性无功能胰岛细胞瘤病例。患者为41岁女性，既往有甲状腺癌（乳头状癌）病史及因甲状旁腺腺瘤导致的甲状旁腺功能亢进。后来，同时发现1例无功能垂体瘤和5例无功能胰腺肿瘤，该患者最终被诊断为MEN I。手术摘除后，胰腺肿瘤经组织病理学诊断为良性胰岛细胞瘤。其中1个肿瘤（肿瘤3）呈实性结节状，其他肿瘤呈脑回状。它们在组织化学和免疫组织化学上分为3种类型：2个肿瘤（肿瘤1和2）分泌单一激素（胰高血糖素），1个肿瘤（肿瘤3）分泌5种激素（胰岛素、胰高血糖素、生长抑素、胃泌素和胰多肽），其余2个肿瘤（肿瘤4和5）分泌2种激素（胰高血糖素和胰多肽）。电镜下，在肿瘤3的肿瘤细胞中发现3种内分泌颗粒，但在肿瘤4中仅发现1种。然而，在肿瘤4提取物中，通过放射免疫测定法检测到了胰高血糖素、胰多肽、C肽、生长抑素、血管活性肠肽和生长激素释放因子。这些发现表明这些胰腺肿瘤具有多细胞性和多激素性。
