Saurenmann P, Binswanger R, Maurer R, Stamm B, Hegglin J
Pathologisches Institut, Stadtspital Triemli, Zürich.
Schweiz Med Wochenschr. 1987 Jul 25;117(30):1134-9.
Somatostatin-producing tumors of the pancreas were first described in 1977. In 1983 a syndrome involving multiple endocrine neoplasias (MEN) was named type III A. This syndrome consists of carcinoid of the duodenum, often producing somatostatin, and von Recklinghausen's disease (neurofibromatosis) or pheochromocytoma. The case is reported of a 62-year-old man with familial neurofibromatosis and a tumor of the head of the pancreas spreading into pars II of the duodenum. After Whipple's duodenopancreatectomy the patient exhibited no further symptoms. Immunohistochemistry served to prove the production of somatostatin and small amounts of calcitonin in the tumor.
胰腺产生生长抑素的肿瘤于1977年首次被描述。1983年，一种涉及多发性内分泌肿瘤（MEN）的综合征被命名为III A型。该综合征由十二指肠类癌（常产生生长抑素）以及冯·雷克林豪森病（神经纤维瘤病）或嗜铬细胞瘤组成。本文报道了一名62岁男性，患有家族性神经纤维瘤病，胰腺头部肿瘤蔓延至十二指肠第二部。在进行惠普尔十二指肠胰腺切除术后，患者未再出现症状。免疫组织化学证实肿瘤中产生生长抑素和少量降钙素。
