Pancreatic involvement in Korean patients with von Hippel-Lindau disease.
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.
Lee
Kwang Hyuck
KH
Department of Internal Medicine, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Gangnam-gu, Seoul, Korea.
Lee
Jae Seung
JS
Kim
Bum Jin
BJ
Lee
Jong Kyun
JK
Kim
Seong Hyun
SH
Kim
Seung Hoon
SH
Lee
Kyu Taek
KT