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Hepatocellular carcinoma occurring in alagille syndrome

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Authors
Kim, Bomi; Park, Sung-Hye; Yang, Hye Ran; Seo, Jeong Kee; Kim, Woo Sun; Chi, Je Geun
Issue Date
2005-02-01
Publisher
Elsevier
Citation
Pathol. Res. Pract. 201 (2005) 55-60
Keywords
Alagille syndromeBile duct diseaseHepatocellular carcinoma
Abstract
Hepatocellular carcinoma only rarely occurs in Alagille syndrome. Here, we report on three cases of hepatocellular carcinoma associated with Alagille syndrome. All three patients were boys and presented with jaundice. In addition, they had the characteristic facial appearance of Alagille syndrome with cardiac, vertebral, and eye anomalies, and all had passed acholic stools from the neonatal period. Liver biopsies were diagnosed as bile duct paucity, compatible with Alagille syndrome in two cases, but the third case showed marked bile duct proliferation at the initial liver biopsy when 7 months old, which made diagnosis difficult. Eventually, all three cases progressed to biliary cirrhosis and hepatocellular carcinoma, which occurred at 17 months, 4 years, and 7 years, respectively. Because of the unusual liver histology and early onset of hepatocellular carcinoma, careful clinicopathologic correlations and close monitoring are required for the diagnosis of Alagille syndrome and for the early detection of hepatocellular carcinoma.
ISSN
0344-0338
Language
English
URI
http://hdl.handle.net/10371/9884
DOI
https://doi.org/10.1016/j.prp.2004.11.007
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College of Medicine/School of Medicine (의과대학/대학원)Pathology (병리학전공)Journal Papers (저널논문_병리학전공)
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