Yamato T, Sasaki M, Hoso M, Sakai J, Ohta H, Watanabe Y, Nakanuma Y
Department of Pathology (II), Kanazawa University School of Medicine, Japan.
J Hepatol. 1998 Apr;28(4):717-22. doi: 10.1016/s0168-8278(98)80297-3.
We report an autopsy case of a 60-year-old woman who had congenital hepatic fibrosis with intrahepatic cholangiocarcinoma. A white nodular lesion with a surrounding vague gray area was detected in the right lobe of the liver. Microscopically, most of the nodular lesion was a poorly-differentiated adenocarcinoma. In the surrounding gray area, small bile ducts and bile ductules showed prominent epithelial overgrowth, some of which was composed of dysplasia and well-differentiated adenocarcinoma. The background liver showed the characteristic features of congenital hepatic fibrosis. Immunohistochemically, biliary oncofetal markers (mucin core protein 1 and carcinoembryonic antigen) were more frequently and extensively expressed in poorly-differentiated than well-differentiated adenocarcinoma. This is the 4th reported case of intrahepatic cholangiocarcinoma arising in congenital hepatic fibrosis and suggests that malignant transformation via dysplasia occurs in the abnormal intrahepatic biliary tree of older congenital hepatic fibrosis patients.
我们报告一例60岁女性尸检病例，该患者患有先天性肝纤维化并伴发肝内胆管癌。在肝脏右叶发现一个白色结节性病变，其周围有模糊的灰色区域。显微镜下，大部分结节性病变为低分化腺癌。在周围的灰色区域，小胆管和细胆管显示出明显的上皮过度生长，其中一些由发育异常和高分化腺癌组成。背景肝脏呈现出先天性肝纤维化的特征性表现。免疫组化显示，与高分化腺癌相比，低分化腺癌中胆管癌胚标志物（粘蛋白核心蛋白1和癌胚抗原）的表达更频繁、更广泛。这是第四例报道的先天性肝纤维化并发肝内胆管癌的病例，提示在年龄较大的先天性肝纤维化患者异常的肝内胆管树中，通过发育异常发生了恶性转化。
