van Kouwen M C, Drenth J P, Engels L G, Strijk S P, van Krieken J H, Nagengast F M
Afd. Maag-, Darm- en Leverziekten, Universitair Medisch Centrum St Radboud, Postbus 9101, 6500 HB Nijmegen.
Ned Tijdschr Geneeskd. 2003 Jul 5;147(27):1323-7.
A 21-year-old man was admitted because of upper abdominal pain and cholestasis. Endoscopic retrograde cholangiopancreatography was suggestive of primary sclerosing cholangitis. During follow-up the patient developed symptoms which were not compatible with primary sclerosing cholangitis, i.e. icterus and weight loss. Finally the patient died, almost three years after presentation, because of a metastatic adenocarcinoma which had arisen from biliary papillomatosis. Biliary papillomatosis is characterised by papillary adenomatous proliferation of the bile duct epithelium. It has a high chance of malignant degeneration. The only curative option would have been transplantation of the liver and biliary system, but this ought to have happened at an early stage before malignant degeneration had occurred.
一名21岁男性因上腹部疼痛和胆汁淤积入院。内镜逆行胰胆管造影提示原发性硬化性胆管炎。在随访过程中，患者出现了与原发性硬化性胆管炎不相符的症状，即黄疸和体重减轻。最终，患者在出现症状近三年后因胆管乳头状瘤病引发的转移性腺癌死亡。胆管乳头状瘤病的特征是胆管上皮的乳头状腺瘤样增生。它有很高的恶变几率。唯一的治愈选择本应是肝和胆道系统移植，但这应该在恶变发生之前的早期进行。
