Acinar Cell Carcinoma of the Pancreas (PAAC)

Overview

Acinar cell carcinoma of the pancreas is a rare malignant pancreatic tumor accounting for ~1–2% of pancreatic exocrine neoplasms, classified under Pancreatic Cancer in OncoTree (parent: PANCREAS). It arises from acinar cells and is distinct from the more common pancreatic ductal adenocarcinoma (PAAD).

Cohorts in the corpus

  • msk_chord_2024: included within the MSK-CHORD pancreatic cancer cohort (n=3,109 PAAD/PAAC combined) with MSK-IMPACT targeted sequencing and NLP-derived clinical annotations. PMID:39506116

Recurrent alterations

  • No PAAC-specific alterations separately reported in MSK-CHORD; the pancreatic cancer cohort (n=3,109) pools PAAD and PAAC. TP53-pathway alterations were associated with higher liver but lower CNS metastasis across pancreatic cancer. PMID:39506116
  • First whole-exome sequencing study of 17 acinar cell carcinomas (plus 3 mixed acinar-ductal and 2 pancreatoblastomas) found mean 119 somatic mutations per tumor, no KRAS mutations, and recurrent alterations in SMAD4 (26%), JAK1 (17%), BRAF (13%), RB1 (13%), and TP53 (13%); >43% carried potentially targetable alterations in the Fanconi anemia pathway (BRCA2, PALB2, ATM, BAP1) or BRAF/JAK1. PMID:24293293
  • GNAS codon 201 hotspot mutations (R201C/R201H) found in all 4 colloid (PAAC) cases in a 109-case microdissected WES cohort; all GNAS-mutant cases were IPMN-derived; 4 of 6 GNAS-mutant cases also harbored KRAS mutations PMID:25855536

Subtypes

  • Pure acinar cell carcinoma
  • Mixed acinar-neuroendocrine carcinoma
  • Mixed acinar-ductal carcinoma

Therapeutic landscape

  • Multimodal random survival forest (RSF) combining NLP-derived tumor sites and treatment features with genomic data outperformed stage-only models for predicting OS in pancreatic cancer (stage IV: P=0.003). PMID:39506116

Sources

  • PMID:39506116 — Gao et al. Nature Medicine 2024. MSK-CHORD clinicogenomic real-world dataset.

This page was processed by crosslinker on 2026-05-14. - PMID:24293293

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

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