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

Autor: Patrick S. Kamath, William P. Martin, Vicente E. Torres, Marie C. Hogan, Peter C. Harris, Walter K. Kremers, Ayush Sharma, Bairbre A. McNicholas, Suresh T. Chari, Naoki Takahashi, Marie E. Edwards, Yoshida Kotaro
Rok vydání: 2019
Předmět:
Adult
Male
medicine.medical_specialty
Endocrinology
Diabetes and Metabolism

Autosomal dominant polycystic kidney disease
urologic and male genital diseases
Gastroenterology
Asymptomatic
Article
Lesion
03 medical and health sciences
0302 clinical medicine
Endocrinology
Internal medicine
Outcome Assessment
Health Care

Internal Medicine
Carcinoma
medicine
Humans
Retrospective Studies
Hepatology
medicine.diagnostic_test
Intraductal papillary mucinous neoplasm
business.industry
Magnetic resonance imaging
Middle Aged
medicine.disease
Polycystic Kidney
Autosomal Dominant

Adenocarcinoma
Mucinous

Magnetic Resonance Imaging
Pancreatic Neoplasms
Adenocarcinoma
Papillary

030220 oncology & carcinogenesis
Mutation
Adenocarcinoma
030211 gastroenterology & hepatology
Female
medicine.symptom
Pancreatic cysts
Pancreatic Cyst
business
Carcinoma
Pancreatic Ductal
Zdroj: Pancreas
ISSN: 1536-4828
Popis: 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.
Databáze: OpenAIRE