Diagnostics and prospective outcome of a diffuse glioneuronal tumor with oligodendroglioma-like features and nuclear clusters after surgical resection (DGONC): a case report

Abstract

Diffuse glioneuronal tumors with oligodendroglioma-like features and nuclear clusters (DGONC) are rare tumors of the central nervous system, having been added as a provi-sional diagnosis in the 2021 World Health Organization (WHO) Classification of Tumors of the central nervous system (CNS).1Retrospective studies report that these are most often mis-diagnosed and treated as high-grade tumors of the CNS.2,3However, this entity has demonstrated superior long-term survival in comparison. Given its novelty, there is currently no standard of care. We describe the diagnostic challenges, mo-lecular characteristics, prospective management, and outcome of a pediatric patient with a DGONC, with the aim to increase awareness of this entity and describe clinical behavior and di-agnostic uncertainties.