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

2 Citations (Scopus)

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

Fingerprint

von Hippel-Lindau Disease
Cysts
Neoplasms
Serous Cystadenoma
Neuroendocrine Tumors
Electronic Health Records
Demography

Keywords

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

ASJC Scopus subject areas

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

Cite this

Clinical Profile of Pancreatic Cystic Lesions in von Hippel-Lindau Disease : A Series of 48 Patients Seen at a Tertiary Institution. / Sharma, Ayush; Mukewar, Saurabh; Vege, Santhi Swaroop.

In: Pancreas, Vol. 46, No. 7, 01.08.2017, p. 948-952.

Research output: Contribution to journalArticle

@article{3f4ef5f00f6947a1b7ff32f2873ddc05,
title = "Clinical Profile of Pancreatic Cystic Lesions in von Hippel-Lindau Disease: A Series of 48 Patients Seen at a Tertiary Institution",
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.",
keywords = "neuroendocrine tumor, pancreatic caner, pancreatic cyst, pancreatic lesions, VHL disease",
author = "Ayush Sharma and Saurabh Mukewar and Vege, {Santhi Swaroop}",
year = "2017",
month = "8",
day = "1",
doi = "10.1097/MPA.0000000000000875",
language = "English (US)",
volume = "46",
pages = "948--952",
journal = "Pancreas",
issn = "0885-3177",
publisher = "Lippincott Williams and Wilkins",
number = "7",

}

TY - JOUR

T1 - Clinical Profile of Pancreatic Cystic Lesions in von Hippel-Lindau Disease

T2 - A Series of 48 Patients Seen at a Tertiary Institution

AU - Sharma, Ayush

AU - Mukewar, Saurabh

AU - Vege, Santhi Swaroop

PY - 2017/8/1

Y1 - 2017/8/1

N2 - 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.

AB - 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.

KW - neuroendocrine tumor

KW - pancreatic caner

KW - pancreatic cyst

KW - pancreatic lesions

KW - VHL disease

UR - http://www.scopus.com/inward/record.url?scp=85025594531&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85025594531&partnerID=8YFLogxK

U2 - 10.1097/MPA.0000000000000875

DO - 10.1097/MPA.0000000000000875

M3 - Article

C2 - 28697137

AN - SCOPUS:85025594531

VL - 46

SP - 948

EP - 952

JO - Pancreas

JF - Pancreas

SN - 0885-3177

IS - 7

ER -