Intraductal Papillary Mucinous Neoplasm (IPMN)

Overview

Intraductal papillary mucinous neoplasm (IPMN) is a cystic precursor lesion of the pancreas with malignant potential. It arises from the ductal epithelium and is characterized by mucin-producing intraductal proliferation. IPMNs can progress to invasive pancreatic ductal adenocarcinoma (PAAD) and are classified by involvement of main duct, branch duct, or mixed type. Within OncoTree, IPMN sits under the pancreatic cancer hierarchy.

Cohorts in the corpus

  • pact_jhu_2011: 8 IPMN cases sequenced by WES with matched normal tissue.

Recurrent alterations

  • Whole-exome sequencing of 8 IPMNs identified RNF43 as the most commonly mutated gene (6/8; 75%), with KRAS codon 12 mutations in 5/8 and GNAS codon 201 mutations in 5/8; RNF43 inactivation converges with MCN on the ubiquitin ligase pathway PMID:22158988.
  • GNAS codon 201 hotspot mutations (R201C/R201H) are enriched in IPMN-derived invasive pancreatic carcinomas: all 6 GNAS-mutant invasive cases in a 109-case WES cohort arose from IPMN precursors; 4 of 6 GNAS-mutant cases also carried KRAS mutations PMID:25855536

Subtypes

  • Main duct and branch duct subtypes differ in malignant potential; both harbor RNF43 and KRAS mutations as key drivers.
  • GNAS codon 201 mutations (R201C/H) appear predominantly in IPMNs relative to MCNs, potentially serving as a differentiating molecular marker.

Therapeutic landscape

  • No targeted therapy currently approved specifically for IPMN; the five-gene panel (VHL, RNF43, CTNNB1, GNAS, KRAS) may serve as a diagnostic tool to distinguish IPMN from benign serous cystadenoma (SCA) in cyst fluid, potentially guiding surgical decision-making PMID:22158988.

Sources

  • PMID:22158988 — WES of 8 IPMN cases defining somatic mutation landscape.

This page was processed by crosslinker on 2026-05-14. - PMID:25855536

This page was processed by crosslinker on 2026-05-14.