Pancreatic Cysts and Intraductal Papillary Mucinous Neoplasm in Autosomal Dominant Polycystic Kidney Disease

Bairbre A. McNicholas, Yoshida Kotaro, William Martin, Ayush Sharma, Patrick Sequeira Kamath, Marie E. Edwards, Walter K Kremers, Suresh T Chari, Vicente Torres, Peter C Harris, Naoki M Takahashi, Marie C Hogan

Research output: Contribution to journalArticle

Abstract

OBJECTIVES: Pancreatic lesions in autosomal dominant polycystic kidney disease (ADPKD) are primarily cysts. They are increasingly recognized, with isolated reports of intraductal papillary mucinous neoplasia (IPMN). METHODS: Retrospective study to determine prevalence, number, size, and location of pancreatic abnormalities using abdominal magnetic resonance imaging (MRI) of genotyped ADPKD patients (seen February 1998 to October 2013) and compared with age- and sex-matched non-ADPKD controls. We evaluated presentation, investigation, and management of all IPMNs among individuals with ADPKD (January 1997 to December 2016). RESULTS: Abdominal MRIs were examined for 271 genotyped ADPKD patients. A pancreatic cyst lesion (PCL) was detected in 52 patients (19%; 95% confidence interval, 15%-23%). Thirty-seven (71%) had a solitary PCL; 15 (28%) had multiple. Pancreatic cyst lesion prevalence did not differ by genotype. Intraductal papillary mucinous neoplasia was detected in 1% of ADPKD cases. Among 12 IPMN patients (7 branch duct; 5 main duct or mixed type) monitored for about 140 months, 2 with main duct IPMNs required Whipple resection, and 1 patient died of complications from small-bowel obstruction after declining surgical intervention. CONCLUSIONS: With MRI, PCLs were detected in 19% and IPMNs in 1% of 271 ADPKD patients with proven mutations, without difference across genotypes. Pancreatic cyst lesions were asymptomatic and remained stable in size.

Original languageEnglish (US)
Pages (from-to)698-705
Number of pages8
JournalPancreas
Volume48
Issue number5
DOIs
StatePublished - May 1 2019

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Pancreatic Cyst
Autosomal Dominant Polycystic Kidney
Neoplasms
Genotype
Magnetic Resonance Imaging
Bone Cysts
Polycystic Kidney Diseases
Cysts
Retrospective Studies
Confidence Intervals
Mutation

ASJC Scopus subject areas

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

Cite this

Pancreatic Cysts and Intraductal Papillary Mucinous Neoplasm in Autosomal Dominant Polycystic Kidney Disease. / McNicholas, Bairbre A.; Kotaro, Yoshida; Martin, William; Sharma, Ayush; Kamath, Patrick Sequeira; Edwards, Marie E.; Kremers, Walter K; Chari, Suresh T; Torres, Vicente; Harris, Peter C; Takahashi, Naoki M; Hogan, Marie C.

In: Pancreas, Vol. 48, No. 5, 01.05.2019, p. 698-705.

Research output: Contribution to journalArticle

McNicholas, Bairbre A. ; Kotaro, Yoshida ; Martin, William ; Sharma, Ayush ; Kamath, Patrick Sequeira ; Edwards, Marie E. ; Kremers, Walter K ; Chari, Suresh T ; Torres, Vicente ; Harris, Peter C ; Takahashi, Naoki M ; Hogan, Marie C. / Pancreatic Cysts and Intraductal Papillary Mucinous Neoplasm in Autosomal Dominant Polycystic Kidney Disease. In: Pancreas. 2019 ; Vol. 48, No. 5. pp. 698-705.
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abstract = "OBJECTIVES: Pancreatic lesions in autosomal dominant polycystic kidney disease (ADPKD) are primarily cysts. They are increasingly recognized, with isolated reports of intraductal papillary mucinous neoplasia (IPMN). METHODS: Retrospective study to determine prevalence, number, size, and location of pancreatic abnormalities using abdominal magnetic resonance imaging (MRI) of genotyped ADPKD patients (seen February 1998 to October 2013) and compared with age- and sex-matched non-ADPKD controls. We evaluated presentation, investigation, and management of all IPMNs among individuals with ADPKD (January 1997 to December 2016). RESULTS: Abdominal MRIs were examined for 271 genotyped ADPKD patients. A pancreatic cyst lesion (PCL) was detected in 52 patients (19{\%}; 95{\%} confidence interval, 15{\%}-23{\%}). Thirty-seven (71{\%}) had a solitary PCL; 15 (28{\%}) had multiple. Pancreatic cyst lesion prevalence did not differ by genotype. Intraductal papillary mucinous neoplasia was detected in 1{\%} of ADPKD cases. Among 12 IPMN patients (7 branch duct; 5 main duct or mixed type) monitored for about 140 months, 2 with main duct IPMNs required Whipple resection, and 1 patient died of complications from small-bowel obstruction after declining surgical intervention. CONCLUSIONS: With MRI, PCLs were detected in 19{\%} and IPMNs in 1{\%} of 271 ADPKD patients with proven mutations, without difference across genotypes. Pancreatic cyst lesions were asymptomatic and remained stable in size.",
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AU - McNicholas, Bairbre A.

AU - Kotaro, Yoshida

AU - Martin, William

AU - Sharma, Ayush

AU - Kamath, Patrick Sequeira

AU - Edwards, Marie E.

AU - Kremers, Walter K

AU - Chari, Suresh T

AU - Torres, Vicente

AU - Harris, Peter C

AU - Takahashi, Naoki M

AU - Hogan, Marie C

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N2 - OBJECTIVES: Pancreatic lesions in autosomal dominant polycystic kidney disease (ADPKD) are primarily cysts. They are increasingly recognized, with isolated reports of intraductal papillary mucinous neoplasia (IPMN). METHODS: Retrospective study to determine prevalence, number, size, and location of pancreatic abnormalities using abdominal magnetic resonance imaging (MRI) of genotyped ADPKD patients (seen February 1998 to October 2013) and compared with age- and sex-matched non-ADPKD controls. We evaluated presentation, investigation, and management of all IPMNs among individuals with ADPKD (January 1997 to December 2016). RESULTS: Abdominal MRIs were examined for 271 genotyped ADPKD patients. A pancreatic cyst lesion (PCL) was detected in 52 patients (19%; 95% confidence interval, 15%-23%). Thirty-seven (71%) had a solitary PCL; 15 (28%) had multiple. Pancreatic cyst lesion prevalence did not differ by genotype. Intraductal papillary mucinous neoplasia was detected in 1% of ADPKD cases. Among 12 IPMN patients (7 branch duct; 5 main duct or mixed type) monitored for about 140 months, 2 with main duct IPMNs required Whipple resection, and 1 patient died of complications from small-bowel obstruction after declining surgical intervention. CONCLUSIONS: With MRI, PCLs were detected in 19% and IPMNs in 1% of 271 ADPKD patients with proven mutations, without difference across genotypes. Pancreatic cyst lesions were asymptomatic and remained stable in size.

AB - OBJECTIVES: Pancreatic lesions in autosomal dominant polycystic kidney disease (ADPKD) are primarily cysts. They are increasingly recognized, with isolated reports of intraductal papillary mucinous neoplasia (IPMN). METHODS: Retrospective study to determine prevalence, number, size, and location of pancreatic abnormalities using abdominal magnetic resonance imaging (MRI) of genotyped ADPKD patients (seen February 1998 to October 2013) and compared with age- and sex-matched non-ADPKD controls. We evaluated presentation, investigation, and management of all IPMNs among individuals with ADPKD (January 1997 to December 2016). RESULTS: Abdominal MRIs were examined for 271 genotyped ADPKD patients. A pancreatic cyst lesion (PCL) was detected in 52 patients (19%; 95% confidence interval, 15%-23%). Thirty-seven (71%) had a solitary PCL; 15 (28%) had multiple. Pancreatic cyst lesion prevalence did not differ by genotype. Intraductal papillary mucinous neoplasia was detected in 1% of ADPKD cases. Among 12 IPMN patients (7 branch duct; 5 main duct or mixed type) monitored for about 140 months, 2 with main duct IPMNs required Whipple resection, and 1 patient died of complications from small-bowel obstruction after declining surgical intervention. CONCLUSIONS: With MRI, PCLs were detected in 19% and IPMNs in 1% of 271 ADPKD patients with proven mutations, without difference across genotypes. Pancreatic cyst lesions were asymptomatic and remained stable in size.

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