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Sohn, Cho, and Park: Pancreatic Cyst in von Hippel-Lindau Disease
Case Report
Journal of the Korean Surgical Society

Journal of the Korean Surgical Society 2009; 77(4): 287-290.

Published online: 30 September 2009

DOI: https://doi.org/10.4174/jkss.2009.77.4.287

Pancreatic Cyst in von Hippel-Lindau Disease

Bo Sung Sohn, M.D., Chung Eun Cho, M.D., Il Young Park, M.D.

Department of Surgery, Holy Family Hospital, The Catholic University of Korea, Bucheon, Korea.

Corresponding author (parkiy@catholic.ac.kr)

Received 21 March 2009       Accepted 13 April 2009

Copyright © 2009 The Korean Surgical Society

Abstract

The von Hippel-Lindau (VHL) disease is an autosomal dominant disorder by germline mutation of VHL tumor suppressor gene. The affected patients mainly develop cancers of the central nervous system, renal cell carcinoma, pheochromocytoma, and pancreatic cysts. Pancreatic involvement was observed 12~77% in VHL patients and included true cysts, serous cystadenomas, and neuroendocrine tumors. We experienced two cases of pancreatic cyst of the von Hippel-Lindau disease and reviewed associated literatures.

Keywords: von Hippel-Lindau disease, Pancreatic cyst

Figures and Tables

Fig. 1
MRI (T2 weighted imaging); cystic mass in cerebellum (arrow).
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Fig. 2
Pedigree of Patients. Mother and two daughters are VHL gene positive. Mother and one daughter have pancreatic cysts. The other daughter has sensory neural hearing loss.
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Fig. 3
Gene study. Mother and two daughters have a mutation of 257 nucleotide.
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Fig. 4
Abdominal CT showed 1 cm sized pancreatic cyst in body and tail (arrow). There are no lesions in liver and spleen.
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Fig. 5
Brain MRI showed there was a cystic tumor in the inferior aspect of Lt. cerebellum by 3×4×3.5 cm size (arrow).
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Fig. 6
Abdominal CT findings are multiple cysts in pancreas head portion diameter 5 cm in size, with internal serous material (black arrow) and distal pancreas portion has multiple cysts (white arrow).
jkss-77-287-g006

References

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