Kert Špela, Matjašič Alenka, Pižem Jože, Mlakar Jernej, Bošnjak Matic, Jerala Miha, Kotnik Primož, Faganel Kotnik Barbara, Kitanovski Lidija, Zupan Andrej
Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Clinical Institute of Genomic Medicine, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Brain Tumor Pathol. 2025 Jul 9. doi: 10.1007/s10014-025-00509-z.
Craniopharyngiomas (CPs) are rare benign brain tumors that are classified as WHO grade I, with two subtypes: adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP). ACP is caused by somatic mutations in exon 3 of the CTNNB1 gene activating the Wnt signaling pathway. PCP is associated with somatic BRAF p.V600E mutations activating the MAPK signaling pathway. Understanding their molecular differences is crucial for diagnosis and treatment. This study aimed to analyze common somatic alterations in ACP and PCP using bulk transcriptome sequencing and in situ spatial transcriptomics. RNA sequencing and high-resolution spatial profiling were used to detect mutations and examine gene expression differences among ACP, PCP, and healthy pituitary tissue. Whole transcriptome sequencing was performed on 24 tumor samples, with healthy pituitary data from the GTEx portal. Bioinformatics analysis utilized the CTAT mutation pipeline, with Sanger sequencing for validation. Results confirmed BRAF p.V600E mutations in all PCP samples and CTNNB1 mutations in all ACP samples. Differential gene expression analysis highlighted distinct molecular profiles and reinforced the involvement of Wnt and MAPK signaling. Spatial profiling identified 41 differentially expressed genes between ACP and PCP. This study provides critical insights into CP biology, supporting improved diagnostics and potential therapeutic strategies.
颅咽管瘤(CPs)是罕见的良性脑肿瘤,被归类为世界卫生组织一级,有两种亚型:造釉细胞瘤型颅咽管瘤(ACP)和乳头型颅咽管瘤(PCP)。ACP由CTNNB1基因第3外显子的体细胞突变引起,激活Wnt信号通路。PCP与激活MAPK信号通路的BRAF p.V600E体细胞突变有关。了解它们的分子差异对诊断和治疗至关重要。本研究旨在使用批量转录组测序和原位空间转录组学分析ACP和PCP中的常见体细胞改变。RNA测序和高分辨率空间分析用于检测突变,并检查ACP、PCP和健康垂体组织之间的基因表达差异。对24个肿瘤样本进行了全转录组测序,并使用了来自GTEx数据库的健康垂体数据。生物信息学分析采用CTAT突变流程,并通过桑格测序进行验证。结果证实所有PCP样本中存在BRAF p.V600E突变,所有ACP样本中存在CTNNB1突变。差异基因表达分析突出了不同的分子特征,并加强了Wnt和MAPK信号通路的参与。空间分析确定了ACP和PCP之间41个差异表达基因。本研究为颅咽管瘤生物学提供了关键见解,有助于改进诊断和潜在的治疗策略。