1型神经纤维瘤病与壶腹神经节细胞性副神经节瘤导致胆管和胰腺梗阻。

Neurofibromatosis-1 and ampullary gangliocytic paraganglioma causing biliary and pancreatic obstruction.

作者信息

Castoldi L, De Rai P, Marini A, Ferrero S, De Luca V M, Tiberio G

机构信息

Institute of Emergency Surgery, University of Milan, Ospedale Maggiore IRCCS, Italy.

出版信息

Int J Pancreatol. 2001;29(2):93-7. doi: 10.1385/IJGC:29:2:093.

DOI:10.1385/IJGC:29:2:093

PMID:11876254

Abstract

Gangliocytic paragangliomas (GPs) are exceedingly rare tumors that arise in close proximity of the papilla of Vater. Nevertheless, jaundice is an uncommon presenting symptom, reported in only 3 of 125 cases described in the literature to date, with gastrointestinal bleeding being more common. Association between GPs and neurofibromatosis 1 (NF-1), described in two patients, may be more than casual, being the frequency in normal population 1:3,000. We report an additional case of this association, presenting for the first time with simultaneous obstruction of the common biliary duct, and of the main pancreatic duct. Previous cases of GPs with jaundice and/or NF-1 are reviewed, and updating of the histogenesis presented.

摘要

神经节细胞副神经节瘤（GPs）是极为罕见的肿瘤，发生于靠近 Vater 壶腹处。然而，黄疸是一种不常见的首发症状，在迄今为止文献报道的 125 例病例中仅有 3 例出现，而胃肠道出血更为常见。文献中描述了 2 例 GPs 与神经纤维瘤病 1 型（NF-1）之间的关联，这种关联可能并非偶然，因为 NF-1 在正常人群中的发病率为 1:3000。我们报告了另外一例这种关联的病例，首次表现为胆总管和主胰管同时梗阻。对既往有黄疸和/或 NF-1 的 GPs 病例进行了回顾，并对组织发生学进行了更新。