胆管拡張部に上皮内胆管細胞癌を合併した Caroli 病の一切除例

概要

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Caroli's disease is a congenital disorder with cystic dilatation of the intrahepatic biliary tract. A 57-year-old woman admitted complaining of epigastralgia. Ultrasonograms showed cystic dilatation of the intrahepatic bile ducts in the left lobe without hepatolithiasis. The illness was diagnosed as Caroli's disease. The patient underwent resection of the left hepatic lobe and caudate lobe. Macroscopic inspection showed no malignancy, but microscopic inspection showed well-differentiated papillary adenocarcinoma in the epithelium.<BR>In cases reported in the literature, Caroli's disease is accompanied with cholangiocellular carcinoma in about 8% of patients. The continuous irritation from stones, long-standing bile stasis, repeated cholangitis, and some congenital factor seem to trigger the development of carcinoma. This theory is generally accepted by surgeons considering treatment of choledochal cysts or an anomalous arrangement of the pancreatobiliary ducts. However, in our case, there was no cystic dilataion of extrahepatic bile ducts and no hepatolithiasis. Regeneration, hyperplasia, and metaplasia of the epithelium of the bile ducts were seen in cancer-free areas of the operative specimen. Hepatic resection should be performed for some patients with Caroli's disease.
社団法人日本肝臓学会の論文