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Kidney Week

Abstract: SA-PO487

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

Session Information

  • ADPKD: Clinical Studies
    October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidney

  • 1001 Genetic Diseases of the Kidney: Cystic

Authors

  • McNicholas, Bairbre A., Galway University Hospitals, Galway, Ireland
  • Martin, William P., Galway University Hospital, Health Service Executive West, Galway , Ireland
  • Edwards, Marie E., Mayo Clinic , Rochester, Minnesota, United States
  • Kamath, Patrick S., Mayo Clinic, Rochester, Minnesota, United States
  • Torres, Vicente E., Mayo Clinic , Rochester, Minnesota, United States
  • Harris, Peter C., Mayo Clinic , Rochester, Minnesota, United States
  • Hogan, Marie C., Mayo Clinic , Rochester, Minnesota, United States
Background

Pancreatic Cyst lesions in ADPKD are primarily cysts. They are recognized increasingly, with isolated reports of intraductal papillary mucinous neoplasia (IPMN).

Methods

Tertiary care center retrospective study to determine prevalence, number, size, and location of pancreatic abnormalities (pancreatic cyst lesions [PCLs], IPMN, and not-otherwise-specified abnormalities) with abdominal magnetic resonance imaging (MRI) of genotyped ADPKD patients (1998 - 2013) and compared with age- and sex-matched non-ADPKD controls. We evaluated presentation, investigation, and management of all IPMNs among individuals with ADPKD (1997- 2016), for natural history and management of pancreatic abnormalities.

Results

Abdominal MRIs (104 with and 167 without gadolinium) were examined for 271 genotyped ADPKD patients. At least 1 PCL was noted in 19% of ADPKD patients (n=52) compared with 10.2% of controls (n=28) (P=.03). Mean (SD) age at diagnosis was 42(12) years for ADPKD vs 50(9) years (controls) (P<.001). Thirty-seven (71%) had a solitary PCL; 15 (28%) had multiple. PCL prevalence did not differ by genotype. IPMNs were detected in 1% of ADPKD cases. Among 12 IPMN patients (7 branch duct; 5 main duct or mixed type) monitored for ~140 months, 2 with main duct IPMN required Whipple resection, and 1 patient died of complications from small-bowel obstruction after declining surgical intervention.

Conclusion

With MRI, PCLs were detected in 19% and IPMNs in 1% of 271 ADPKD patients with proven mutations, without difference across genotypes. PCLs were asymptomatic and remained stable in size. No malignant transformation occurred among 12 IPMN patients.