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Pancreatic involvement in Korean patients with von Hippel-Lindau disease

  • Original Article—Liver, Pancreas, and Biliary Tract
  • Published:
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Abstract

Purpose

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.

Conclusion

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.

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Acknowledgments

This work was supported by a Samsung Biomedical Research Institute, grant no. SBRI C-A7- 219-2.

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Authors and Affiliations

  1. Department of Internal Medicine, Samsung Medical Center, School of Medicine, Sungkyunkwan University, #50, Irwon-dong, Gangnam-gu, Seoul, 135-710, Korea

    Kwang Hyuck Lee, Jae Seung Lee, Bum Jin Kim, Jong Kyun Lee & Kyu Taek Lee

  2. Department of Radiology, Samsung Medical Center, School of Medicine, Sungkyunkwan University, Seoul, 135-710, Korea

    Seong Hyun Kim & Seung Hoon Kim

Authors
  1. Kwang Hyuck Lee
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  2. Jae Seung Lee
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  3. Bum Jin Kim
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  4. Jong Kyun Lee
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  5. Seong Hyun Kim
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  6. Seung Hoon Kim
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  7. Kyu Taek Lee
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Correspondence to Kyu Taek Lee.

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Lee, K.H., Lee, J.S., Kim, B.J. et al. Pancreatic involvement in Korean patients with von Hippel-Lindau disease. J Gastroenterol 44, 447–452 (2009). https://doi.org/10.1007/s00535-009-0021-2

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  • DOI: https://doi.org/10.1007/s00535-009-0021-2

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