We identify recurrent, low-VAF IDH1/2 mutations in angiosarcoma and provide evidence that these subclonal mutations promote tumorigenesis through non-cell-autonomous mechanisms. Vascular tumors driven by subclonal IDH1 mutations responded dramatically to ivosidenib, thus revealing a novel treatment for a subset of vascular tumors.
These preliminary data might indicate that targeted therapies should be introduced in first line with different strategies depending on molecular alterations, with access to platinum-based palliative systemic anti-cancer treatment being important for patients with IDH1-mutated BTC.
The recent INDIGO trial, which demonstrated efficacy of the IDH1/2 inhibitor vorasidenib in residual grade 2 IDH-mutant glioma, has further increased the importance of preoperative imaging assessment of IDH mutation status...Third, we discuss the implications of cIMPACT-NOW Updates 8-11 for neuroradiologic diagnosis. In the WHO CNS5 era, imaging plays a central role in supporting integrated diagnosis and prioritizing appropriate molecular testing.