Pancreatic Neuroendocrine Carcinoma (PANEC)
Overview
Pancreatic Neuroendocrine Carcinoma (PANEC) is a poorly differentiated, high-grade neuroendocrine neoplasm of the pancreas under the Pancreatic Cancer parent in OncoTree. Distinguished from well-differentiated pancreatic neuroendocrine tumors (PANET) by high Ki-67 and aggressive clinical behavior.
Cohorts in the corpus
- pog570_bcgsc_2020 — pancreatic NEN patients (PanNENs, including high-grade cases) within 28-patient metastatic NEN WGTA cohort at BC Cancer POG. PMID:24326773
Recurrent alterations
- Whole-genome and transcriptome analysis (WGTA) of metastatic pancreatic NENs confirmed recurrent MEN1, DAXX/ATRX, RB1, and TP53 alterations; high-grade cases clustered in transcriptome Cluster B with enriched MYC-target gene sets. Median TMB was 2.19 mut/Mb across the 28-patient NEN cohort. Actionable alterations were identified in the majority of cases. PMID:24326773
Subtypes
- Poorly differentiated NEC vs. NET-G3; NECs and NET-G3s did not form a distinct transcriptome cluster from low-grade NETs in this study. PMID:24326773
Therapeutic landscape
- MTOR inhibitors, somatostatin analogs (guided by SSTR expression), DLL3 inhibitors, and PARP inhibitors flagged as actionable in PanNEN cases via WGTA. PMID:24326773
Sources
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