Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
ORIGINAL ARTICLES
Imaging Findings of Pancreatic Cystic Lesions in von Hippel-Lindau Disease
Noritoshi Kobayashi, Takamitsu Sato, Shingo Kato, Seitaro Watanabe, Kunihiro Hosono, Takeshi Shimamura, Hiroshi Iida, Hiroki Endo, Tomoko Koide, Hirokazu Takahashi, Masato Yoneda, Wataru Shibata, Yasunobu Abe, Masahiko Inamori, Hiroyuki Kirikoshi, Satoru Saito, Shin Maeda, Atsushi Nakajima, Kensuke Kubota
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Keywords: von Hippel-Lindau disease, pancreatic cysts, neuroendocrine tumors, serous cystic neoplasms
JOURNAL OPEN ACCESS

2012 Volume 51 Issue 11 Pages 1301-1307

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Abstract

Objectives The aim of this study was to characterize the pancreatic cystic lesions in von Hippel-Lindau (VHL) disease and to document the changes that occur in the pancreas.
Methods We retrospectively analyzed the medical records and the computed tomography (CT) and magnetic resonance imaging (MRI) findings of 20 VHL patients who were diagnosed between 1996 and 2010 at our hospital. The clinical findings, family history and type of tumors and/or cysts were reviewed for each patient. We also analyzed the imaging findings for the pancreas in detail.
Results Pancreatic involvement was noted in 16 of the 20 patients (80%). Eleven patients had multiple cysts diffusely distributed in the pancreas, and one patient had a single cyst in the pancreas head. Two patients had serous cystic neoplasms (SCNs) with multiple cysts, and another two patients had neuroendocrine tumors (NETs) which were conventional radiological findings. The largest cysts of four patients (26.7%) increased in size and that of three patients (20%) decreased in size during the follow-up period. We performed surgical resections for the pancreatic tumors (one NET and one SCN) and also performed endoscopic treatment for a pancreatic cyst in one VHL patient with obstructive jaundice. None of the patients died as a result of pancreatic disease.
Conclusion The most common type of pancreatic lesions was multiple cysts. SCNs were present in only 10% of the VHL patients. Pancreatic cysts showed positive and/or negative growth according to the CT and MRI findings. The pancreatic cystic lesions did not influence the outcome of the VHL patients.

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© 2012 by The Japanese Society of Internal Medicine
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