title: Pancreatic involvement in Korean patients with von Hippel-Lindau disease.
abstract: OBJECTIVE
  The aim of this study was to describe pancreatic involvement in von Hippel-Lindau (VHL) disease and to document the changes that occur in pancreatic lesions.
  
  
  METHODS
  We retrospectively analyzed the medical records and CT scans of 18 VHL patients who were diagnosed between 1994 and 2007 at the Samsung Medical Center. The clinical history with a detailed family history, biochemical test results, and imaging studies of the pancreas, adrenal glands, and kidneys were reviewed. Genetic analysis was performed in 12 patients. The changes in pancreatic lesions, such as an increase in cystic lesions, calcifications, and dilatation of the pancreatic duct, were analyzed in patients who had CT scans at least 1 year apart.
  
  
  RESULTS
  Pancreatic lesions existed in 89% (16/18) of the patients. All 16 patients had multiple cystic lesions. Two patients had co-existing neuroendocrine tumors (NET), and two patients had co-existing serous cystadenomas (SCA). At least one of three features of pancreatic lesions (cystic lesions, calcifications, and dilatation of the pancreatic duct) progressed in all nine patients who had CT scans 1 year apart.
  
  
  CONCLUSIONS
  Pancreatic involvement in VHL disease was relatively common in Korean patients. The most common type of pancreatic involvement was a multiple cystic lesion. NET and SCA existed in approximately 10% of VHL patients with pancreatic involvement. Pancreatic lesions in VHL disease progressed, at least according to radiological images.
authors.affiliation: Department of Internal Medicine, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Gangnam-gu, Seoul, Korea.
authors.forename: Kwang Hyuck
authors.initials: KH
authors.lastname: Lee
authors.forename: Jae Seung
authors.initials: JS
authors.lastname: Lee
authors.forename: Bum Jin
authors.initials: BJ
authors.lastname: Kim
authors.forename: Jong Kyun
authors.initials: JK
authors.lastname: Lee
authors.forename: Seong Hyun
authors.initials: SH
authors.lastname: Kim
authors.forename: Seung Hoon
authors.initials: SH
authors.lastname: Kim
authors.forename: Kyu Taek
authors.initials: KT
authors.lastname: Lee