title: Pancreatic involvement in 11 cases of Von Hippel-Lindau disease.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
authors.affiliation: Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Okayama, Japan.
authors.forename: Masaya
authors.initials: M
authors.lastname: Iwamuro
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Kawamoto
authors.forename: Hidenori
authors.initials: H
authors.lastname: Shiraha
authors.forename: Soichiro
authors.initials: S
authors.lastname: Nose
authors.forename: Kazuhide
authors.initials: K
authors.lastname: Yamamoto
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000097:blood; D001786:Blood Glucose / Q000032:analysis; D003920:Diabetes Mellitus / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D005260:Female; D005951:Glucose Tolerance Test; D006785:Hospitals, University; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D010181:Pancreatic Cyst / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000453:epidemiology* / Q000628:therapy
chemical_list: D015415:Biomarkers; D001786:Blood Glucose