Co-occurrence of pediatric-type diffuse high-grade gliomas with arteriovenous malformations: case report and narrative literature review.

PURPOSE: The co-occurrence of pediatric-type diffuse high-grade glioma (pDHGG) and cerebral arteriovenous malformation (AVM) is exceptionally rare. This report describes the youngest documented case of this dual pathology and presents a narrative review of previously published cases to contextualize the management approach.

METHODS

A 14-year-old male presenting with progressive focal seizures and altered consciousness was evaluated with multimodal neuroimaging including MRI, CTA, and DSA. A staged approach was employed: preoperative endovascular embolization using N-butyl cyanoacrylate (NBCA) followed by complete microsurgical en bloc resection under intraoperative neurophysiological monitoring. Histopathological and molecular analyses were performed according to WHO 2021 criteria. A narrative review of previously reported cases was conducted through PubMed/MEDLINE search up to April 2025.

RESULTS

Preoperative embolization achieved approximately 80% reduction in AVM blood flow, facilitating complete en bloc resection of both lesions. Molecular profiling confirmed pDHGG (WHO grade 4, H3-wildtype, IDH-wildtype) with GFAP( +), Olig-2( +), retained ATRX, and Ki-67 ~ 40%. Adjuvant chemoradiotherapy (Stupp protocol) was administered postoperatively. The patient achieved complete neurological recovery and remains disease-free at 24-month follow-up.

CONCLUSION

This case demonstrates the feasibility of a staged preoperative embolization combined with complete microsurgical resection and adjuvant chemoradiotherapy for concurrent pDHGG and AVM. While the favorable outcome is encouraging, larger studies are needed to establish the optimal management strategy for these rare dual pathologies.