Vezzadini P, Poggioli R, Vezzadini C, Alberani A, Dal Monte P R
Dipartimento di Medicina Interna e Gastroenterologia, Ospedale Bellaria, Università degli Studi, Bologna.
Minerva Med. 1996 Jul-Aug;87(7-8):363-8.
This case report describes a 27-year-old man with von Recklinghausen's neurofibromatosis, manifested as cutaneous cafè au lait spots and neurofibromas, associated with duodenal somatostatinoma. The patient presented with ultrasonographic evidence of dilatation of the biliary and pancreatic ducts, in absence of clinical symptoms. The reason for the performance of ultrasonography was to identify the cause of an increase of hepatic enzymes during the last two years. Diagnostic ERCP showed an ulcerated tumor in the papillary region and pathological findings were compatible with somatostatinoma. Endoscopic sphincterotomy with placement of endoprostheses was successful in achieving biliary and pancreatic drainage. Subsequently a curative resection of the tumor was performed by the Whipple procedure and provocative tests demonstrated normal plasma somatostatin concentrations.
本病例报告描述了一名27岁患有冯雷克林霍增氏神经纤维瘤病的男性，表现为皮肤牛奶咖啡斑和神经纤维瘤，并伴有十二指肠生长抑素瘤。患者超声检查显示胆管和胰管扩张，但无临床症状。进行超声检查的原因是为了确定过去两年肝酶升高的原因。诊断性内镜逆行胰胆管造影（ERCP）显示乳头区域有一个溃疡性肿瘤，病理结果与生长抑素瘤相符。内镜括约肌切开术并放置内支架成功实现了胆管和胰管引流。随后通过惠普尔手术对肿瘤进行了根治性切除，激发试验显示血浆生长抑素浓度正常。
