Document Detail

Pancreatic involvement in 11 cases of von hippel-lindau disease.
MedLine Citation:
PMID:  22353527     Owner:  NLM     Status:  In-Data-Review    
Background/Aims: Von Hippel-Lindau disease is an autosomal dominant genetic disorder characterized by neoplasms developing in multiple organs. Although the pancreas is one of the most frequently involved organs, the frequency of pancreatic cysts, cystadenomas, neuroendocrine tumors and diabetes has not been sufficiently evaluated due to the low prevalence of this disease. In this paper, we review and retrospectively analyze 11 patients with von Hippel-Lindau disease. Methodology: Eleven patients (6 males, 5 females) who underwent CT or MRI scans at Okayama University Hospital between 2002 and 2009 were enrolled in this study. Their pancreatic CT scans, MRI scans, biochemical test results and clinical histories were retrospectively reviewed. Results: All patients had one or more pancreatic involvements. Nine of the 11 patients had multiple pancreatic cysts, 2 had dilatation of the main pancreatic duct, 3 had a non-functioning pancreatic endocrine tumor (one patient required pancreatoduodenectomy due to the endocrine carcinoma) and 3 had diabetes mellitus. Pancreatic cystadenomas were not detected in this case series. Conclusions: The prevalence of pancreatic involvement was 100% in this study. Regular screening and scheduled follow-up for pancreatic lesions and diabetes should be performed on individuals predisposed to von Hippel-Lindau disease.
Masaya Iwamuro; Hirofumi Kawamoto; Hidenori Shiraha; Soichiro Nose; Kazuhide Yamamoto
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Hepato-gastroenterology     Volume:  59     ISSN:  0172-6390     ISO Abbreviation:  Hepatogastroenterology     Publication Date:    2012 Mar-Apr
Date Detail:
Created Date:  2012-02-22     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8007849     Medline TA:  Hepatogastroenterology     Country:  Greece    
Other Details:
Languages:  eng     Pagination:  589-91     Citation Subset:  IM    
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