NF1

This retrospective study investigated the clinical, surgical, and molecular characteristics and outcomes of 52 adult patients with H3 K27-altered diffuse midline glioma (DMG) treated with surgical resection. The median overall survival was 17.8 months. Genomic profiling revealed a distinct molecular landscape dominated by H3F3A mutations, frequently co-occurring with TP53 mutations, and a significant enrichment of mutations within the RTK/RAS/PI3K signaling pathway, involving NF1, FGFR1, and PIK3CA. Postoperative adjuvant radiotherapy combined with temozolomide emerged as the sole independent protective factor for overall survival. While maximal safe resection is considered essential, molecular targeted therapies did not achieve independent statistical significance.

Clear cell adenocarcinoma of the urinary tract (CCA-UT) is a rare and aggressive tumor with limited understanding of its clinicopathologic and molecular features. This multi-institutional study characterized 35 cases, showing a female predominance and advanced stage at presentation. Genomic alterations were found in 91% of cases, frequently involving chromatin modifiers such as ATRX, KMT2C, ARID1A, and ARID1B. The heterogeneous molecular profile of these tumors highlights the critical role of molecular analysis in identifying potential therapeutic targets.