Clinical Profile of Pancreatic Cystic Lesions in von Hippel-Lindau Disease: A Series of 48 Patients Seen at a Tertiary Institution

Ayush Sharma, Saurabh Mukewar, Santhi Swaroop Vege

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

Objectives Little is known about the association between pancreatic cystic lesions (PCLs) with von Hippel-Lindau disease (VHLD). In this study, we describe the clinical presentation, type of PCLs, and risk for malignancy in PCLs in VHLD. Methods Patients given a diagnosis of both VHLD and PCLs were identified from electronic medical records at Mayo Clinic, Rochester, MN, from January 1, 2000, to January 1, 2016. Various demographic, clinical, and radiologic variables were recorded. Results Forty-eight patients were identified with PCLs and VHLD. Most were female (73%), and mean (standard deviation) age was 35.9 (14.2) years. Most (92%) were asymptomatic. PCLs included simple cysts (n = 34, 69%), serous cystadenoma (n = 14, 29%), branch duct intraductal papillary neoplasm (n = 5, 10%), and cystic neuroendocrine tumors (n = 2, 4%). Eight (19%) had mixed PCLs. Cyst aspiration was performed in 8 (53%) patients who underwent EUS, and all were negative for malignancy. At a median follow-up of 84 months, no PCL-Associated cancers were seen. Conclusions Simple cyst is the most common type of PCL found in VHL disease; however, other PCLs were also seen with no malignancy potential. Branch duct intraductal papillary mucinous neoplasms were present in 10%, and this association was not hitherto reported.

Original languageEnglish (US)
Pages (from-to)948-952
Number of pages5
JournalPancreas
Volume46
Issue number7
DOIs
StatePublished - Aug 1 2017

Keywords

  • neuroendocrine tumor
  • pancreatic caner
  • pancreatic cyst
  • pancreatic lesions
  • VHL disease

ASJC Scopus subject areas

  • Internal Medicine
  • Endocrinology, Diabetes and Metabolism
  • Hepatology
  • Endocrinology

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