Pancreatoblastoma (PB)
Overview
Pancreatoblastoma is a rare malignant pancreatic neoplasm that sits under Pancreatic Cancer in OncoTree (parent: PANCREAS). It is most common in children and carries a distinct molecular profile from acinar cell carcinomas, featuring CTNNB1/Wnt-beta-catenin pathway activation as a hallmark.
Cohorts in the corpus
- paac_jhu_2014 — 2 pancreatoblastomas within a 23-sample cohort of pancreatic neoplasms with acinar differentiation. PMID:24293293
Recurrent alterations
- Two pancreatoblastomas in a whole-exome sequencing cohort of 23 pancreatic neoplasms with acinar differentiation carried CTNNB1 mutations (present in 2/2 pancreatoblastomas, 0/21 acinar cell carcinomas) and markedly lower mutation burdens (18 and 17 somatic mutations vs mean 131 for acinar cell carcinomas), reinforcing CTNNB1/Wnt-beta-catenin activation as a defining molecular feature. PMID:24293293
- PIPseq cohort: UGT1A1 homozygous *28 (TA)7TAA allele identified in a pineoblastoma patient as pharmacogenomic flag for irinotecan/SN-38 toxicity; DICER1 frameshift L1603Pfs identified as germline flag for Sertoli-Leydig tumor risk (DICER1 syndrome) PMID:28007021
Subtypes
- Pure pancreatoblastoma vs. mixed variants not explicitly subtyped in this cohort.
Therapeutic landscape
- No pancreatoblastoma-specific drug data in the current corpus.
Sources
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