Heymann M F, Moreau A, Chetritt J, Murat A, Leborgne J, Le Neel J C, Visset J, Le Bodic M F
Hôpital G. & R. Laennec, Nantes.
Ann Pathol. 1996;16(3):167-73.
The Multiple Endocrine Neoplasia (MEN I) or Wermer's syndrome is an uncommon disease which is most often inherited and affects mainly parathyroid glands, pancreatic islets and pituitary gland. The aim of this study concerning 61 pancreatic tumors in 16 patients suffering from MEN I was to define the macroscopic, histological and immunohistochemical characteristics of these tumors. The pancreatic endocrine tumors as part of the MEN I syndrome concern multiple tumors of small size, localized most often to the pancreas's tail. In 79% of cases, these tumors have a different predominating peptidic hormonal secretion in a same patient though most of them have plurihormonal secretions. The pancreatic polyendocrinopathy detection imposes a family investigation to look for a type I polyendocrinopathy.
多发性内分泌腺瘤病1型（MEN I）或韦尔默综合征是一种罕见疾病，多为遗传性，主要影响甲状旁腺、胰岛和垂体。本研究针对16例患有MEN I的患者的61个胰腺肿瘤，旨在明确这些肿瘤的大体、组织学和免疫组化特征。作为MEN I综合征一部分的胰腺内分泌肿瘤涉及多个小肿瘤，最常位于胰腺尾部。在79%的病例中，这些肿瘤在同一患者体内具有不同的主要肽类激素分泌，尽管大多数肿瘤具有多种激素分泌。胰腺多内分泌病的检测需要进行家族调查以寻找I型多内分泌病。
