NCCN Guidelines for Treatment of H3 K27M-Mutant Diffuse Glioma Updated
The National Comprehensive Cancer Network (NCCN; Plymouth Meeting, PA) has included Modeyso (dordaviprone; Jazz Pharmaceuticals, Dublin, Ireland) in its Clinical Practice Guidelines in Oncology as a category 2A recommended treatment option for adults and children with recurrent or progressive diffuse high-grade glioma harboring an H3 K27M mutation. The decision follows the Food and Drug Administration’s (FDA) accelerated approval of Modeyso in August 2025, marking the first approved therapy for this rare and aggressive brain tumor.
The NCCN Clinical Practice Guidelines in Oncology provide standardized, expert-driven recommendations that inform cancer care decisions globally. The inclusion of Modeyso as a category 2A recommendation means that there was a uniform NCCN consensus (≥85%) that treatment with the drug is appropriate, with the recommendation being based on lower-level evidence. As a result, health care providers now have an evidence-based option for treating patients with H3 K27M-mutant diffuse glioma.
“The rapid addition of Modeyso to the NCCN Guidelines – in both the Pediatric Central Nervous System Cancers and Central Nervous System Cancers guidelines – reflects the urgency of the unmet need that patients are faced with when diagnosed with this devastating and aggressive brain tumor,” said Kelvin Tan, MB BCh, MRCPCH, Chief Medical Affairs Officer at Jazz Pharmaceuticals. “We are proud to bring Modeyso to patients in the US as the first treatment option for recurrent H3 K27M-mutant diffuse midline glioma, representing a meaningful shift in the treatment landscape for patients and their families.”
Source: Jazz Pharmaceuticals. Modeyso™ (dordaviprone) included in National Comprehensive Cancer Network® (NCCN®) Clinical Practice Guidelines in Oncology for H3 K27M-mutant diffuse glioma. PRNMedia.PRNewswire.com. Published 9 September, 2025. Accessed 9 September, 2025. https://prnmedia.prnewswire.com/news-releases/modeyso-dordaviprone-included-in-national-comprehensive-cancer-network-nccn-clinical-practice-guidelines-in-oncology-for-h3-k27m-mutant-diffuse-glioma-302549794.html