Document Detail


Cowden syndrome complicated with hepatocellular carcinoma possibly originating from non-alcoholic steatohepatitis (NASH).
MedLine Citation:
PMID:  21269388     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
There is currently no report that has documented hepatocellular carcinoma (HCC) in a case of Cowden syndrome. Here, we present the first reported case of HCC in a 60-year-old female patient with Cowden syndrome. We diagnosed the patient using a pathognomonic criterion of the International Cowden Consortium Operational Diagnostic Criteria and performed genetic analysis. Enhanced computed tomography demonstrated a hypervascular tumor in segment VII of the liver. The patient was diagnosed with Cowden syndrome because her mucocutaneous lesions met the pathognomonic criterion. Mutational analysis confirmed a heterozygous germ line TGT→TAT transition at nucleotide 407 in exon 5 of the phosphatase and tensin homolog detected on the chromosome 10 (PTEN) gene. Needle biopsy showed a poorly differentiated HCC. We also diagnosed non-alcoholic steatohepatitis (NASH) from hepatic histological findings of Mallory's bodies and ballooning cells. PTEN-deficient mice reportedly develop HCC through NASH. This is the first reported case of Cowden syndrome complicated with HCC possibly originating from NASH.
Authors:
Takaaki Sugihara; Mari Mandai; Masahiko Koda; Tomomitsu Matono; Takakazu Nagahara; Masaru Ueki; Yoshikazu Murawaki
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Hepatology research : the official journal of the Japan Society of Hepatology     Volume:  41     ISSN:  1386-6346     ISO Abbreviation:  Hepatol. Res.     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-01-28     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9711801     Medline TA:  Hepatol Res     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  189-93     Citation Subset:  -    
Copyright Information:
© 2011 The Japan Society of Hepatology.
Affiliation:
Division of Medicine and Clinical Science, Department of Multidisciplinary Internal Medicine, Faculty of Medicine, Tottori University, Yonago, Japan.
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