Pancreatic involvement in 11 cases of Von Hippel-Lindau disease

Masaya Iwamuro, Hirofumi Kawamoto, Hidenori Shiraha, Soichiro Nose, Kazuhide Yamamoto

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)589-591
Number of pages3
JournalHepato-gastroenterology
Volume59
Issue number114
DOIs
Publication statusPublished - Mar 1 2012

Keywords

  • Diabetes mellitus
  • Neuroendocrine tumor
  • Pancreatic cyst
  • Pancreatic neoplasms
  • Von Hippel-Lindau disease

ASJC Scopus subject areas

  • Hepatology
  • Gastroenterology

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