NDLI: Monolobar hepatobiliary fibropolycystic disease.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Terada, Tadashi Moriki, Toshiaki
Description	Country affiliation: Japan Author Affiliation: Terada T ( Department of Pathology, Shizuoka City Shimizu Hospital, Miyakami 1231, Shizuoka 424-8636, Japan. piyo0111jp@yahoo.co.jp)
Abstract	We herein report a case of monolobar hepatobiliary fibropolycystic disease. A 75-year-old woman presented with heartburn. Imaging modalities including US, CT, and MRI revealed marked atrophy and multiple biliary cysts of the hepatic left lobe. The hepatic right lobe was normal. ERCP and bile duct endoscopy revealed anomalous pancreaticobiliary union, choledochal dilation, dilation of left intrahepatic bile ducts, and small choledochal non-invasive adenocarcinoma. Polycystic kidney diseases were absent. The patient underwent pancreatico-duodenectomy and extended hepatic left lobectomy. Grossly, the hepatic left lobe was markedly atrophic, and studded with numerous biliary cysts. The left intrahepatic bile ducts were dilated (Caroli's disease) and the common bile duct showed type I choledochal dilation. The right hepatic lobe was normal. Histologically, the hepatic left lobe was replaced by fibroelastosis. The intrahepatic bile ducts showed ductal plate malformation such as irregular contours, invaginations, and protrusions. The numerous biliary cysts also showed ductal plate malformation. There were numerous persistent ductal plates and microhamartomas. Many hyalinized destructive biliary cysts and ductal plates were recognized. The liver parenchyma was scant and free of hepatocellular malformations. The portal veins showed old obliterative portal thrombosis. The right hepatic lobe was normal. Immunohistochemically, the biliary cells were positive for cytokeratin 7, 8, 18 and 19, and MUC6 and CD10, but negative for MUC2 and MUC5AC. The biliary cysts, persistent ductal plate, and microhamartomas were positive for fetal apomucin antigen MUC1.
File Format	HTM / HTML
ISSN	12194956
Issue Number	1
Volume Number	17
e-ISSN	15322807
Journal	Pathology & Oncology Research
Language	English
Publisher	Springer
Publisher Date	2011-03-01
Publisher Place	Netherlands
Access Restriction	Subscribed
Subject Keyword	Discipline Oncology Bile Ducts Abnormalities Pathology Caroli Disease Liver Aged Female Hepatectomy Humans Case Reports Journal Article
Content Type	Text
Resource Type	Case study Article
Subject	Cancer Research Pathology and Forensic Medicine Oncology

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