Mucinous Cystic Neoplasm (MCN)
Overview
Mucinous cystic neoplasm (MCN) is a cystic pancreatic tumor that almost exclusively affects women and is characterized by an ovarian-type stromal layer surrounding mucin-producing epithelium. MCNs do not communicate with the pancreatic ductal system (distinguishing them from IPMNs) and carry malignant potential with the risk of progression to invasive pancreatic ductal adenocarcinoma (PAAD).
Cohorts in the corpus
- pact_jhu_2011: 8 MCN cases sequenced by WES with matched normal tissue.
Recurrent alterations
- Whole-exome sequencing of 8 MCNs identified KRAS codon 12 mutations in 6/8 (75%), RNF43 inactivating mutations in 3/8 (38%), and TP53 mutations in 2/8; MCNs and IPMNs share RNF43 and KRAS as co-drivers, suggesting a convergent ubiquitin ligase pathway defect PMID:22158988.
Subtypes
- Histologically graded as low, intermediate, or high-grade dysplasia; TP53 mutations may mark those at highest risk of progression to invasive carcinoma.
Therapeutic landscape
Sources
- PMID:22158988 — WES of 8 MCN cases defining somatic mutation landscape.
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