腺泡细胞癌（ACC）和胰母细胞瘤（PBL）是罕见的伴有腺泡分化的胰腺恶性肿瘤。 ACC 和 PBL 的蛋白质基因组分析揭示了与胰腺导管腺癌 (PDAC) 和良性胰腺相比不同的蛋白质表达模式。 ACC 和 PBL 表现出相似性，富集与 RNA 加工、染色体组织和线粒体相关的蛋白质，而 PDAC 过表达与基于肌动蛋白的过程、细胞外基质和免疫活性基质相关的蛋白质。这些疾病之间在代谢适应、上皮间质转化和 DNA 修复方面的通路活性差异进行了表征。 PBL 显示 Wnt-CTNNB1 和 IGF2 通路上调。 17 个 ACC 特异性蛋白表明与线粒体功能障碍的代谢疾病有关，而 34 个 PBL 特异性蛋白则用胚胎干细胞表型以及染色体蛋白和细胞周期的改变来标记这种儿科癌症。这项研究为 ACC 和 PBL 的蛋白质组学景观提供了新颖的见解，为诊断和治疗开发提供了潜在的目标。© 2024。作者。

Acinar cell carcinoma (ACC) and pancreatoblastoma (PBL) are rare pancreatic malignancies with acinar differentiation. Proteogenomic profiling of ACC and PBL revealed distinct protein expression patterns compared to pancreatic ductal adenocarcinoma (PDAC) and benign pancreas. ACC and PBL exhibited similarities, with enrichment in proteins related to RNA processing, chromosome organization, and the mitoribosome, while PDACs overexpressed proteins associated with actin-based processes, extracellular matrix, and immune-active stroma. Pathway activity differences in metabolic adaptation, epithelial-to-mesenchymal transition, and DNA repair were characterized between these diseases. PBL showed upregulation of Wnt-CTNNB1 and IGF2 pathways. Seventeen ACC-specific proteins suggested connections to metabolic diseases with mitochondrial dysfunction, while 34 PBL-specific proteins marked this pediatric cancer with an embryonic stem cell phenotype and alterations in chromosomal proteins and the cell cycle. This study provides novel insights into the proteomic landscapes of ACC and PBL, offering potential targets for diagnostic and therapeutic development.© 2024. The Author(s).