Department of Pathology, Shizuoka City Shimizu Hospital, Shizuoka, Japan.
Pathol Int. 2010 May;60(5):407-12. doi: 10.1111/j.1440-1827.2010.02535.x.
We herein report a unique monolobar hepatic disease composed of Caroli's disease, peribiliary cysts, ductal plate malformations, peribiliary gland proliferation, hepatolithiasis, and portal phlebosclerosis with thrombi. A 73-year-old man underwent abdominal imaging, which revealed multiple segmental dilations of the left intrahepatic bile ducts. Polycystic kidney diseases were absent. Intrahepatic cholangiocarcinoma was suspected, and extended left lobectomy of the liver was preformed. Grossly, the hepatic left lobe was atrophic, and partly replaced by fibrous tissue. The intrahepatic bile ducts were dilated (Caroli's disease), and showed small calcium bilirubinate hepatoliths. Microscopically, the intrahepatic bile duct showed non-obstructive segmental dilations (Caroli's disease), numerous peribiliary cysts, numerous ductal plate malformations, proliferation of intrahepatic peribiliary glands, and calcium bilirubinate hepatolithiasis. Portal veins showed phlebosclerosis with thrombi. Immunohistochemically, the various biliary epithelial cells were positive for cytokeratin (CK) 7, 8, 18, and 19, and for MUC6 and CD10. They were negative for MUC2 and MUC5AC. The ductal plate malformations were positive for fetal biliary antigen MUC1, but other biliary cell types were negative for MUC1. The present case resembles 'monolobar Caroli's disease'. We believe that the present monolobular liver disease was congenital in origin.
我们在此报告一例独特的单叶性肝疾病，其由 Caroli 病、胆周囊肿、胆管板畸形、胆周腺增生、胆石症和门静脉血栓形成组成。一名 73 岁男性接受了腹部影像学检查，结果显示左肝内胆管多处节段性扩张。未发现多囊肾病。怀疑为肝内胆管细胞癌，并进行了左半肝扩大切除术。大体上，肝左叶萎缩，部分被纤维组织取代。肝内胆管扩张（Caroli 病），并可见小的胆红素钙胆石。镜下，肝内胆管呈非阻塞性节段性扩张（Caroli 病），有许多胆周囊肿、许多胆管板畸形、肝内胆周腺增生和胆红素钙胆石症。门静脉呈血栓性静脉纤维化。免疫组化染色显示，各种胆管上皮细胞 CK7、8、18 和 19、MUC6 和 CD10 阳性，而 MUC2 和 MUC5AC 阴性。胆管板畸形对胎儿胆道抗原 MUC1 阳性，但其他胆管细胞类型对 MUC1 阴性。本例类似于“单叶性 Caroli 病”。我们认为，目前的单叶性肝病是先天性的。
