Astroblastoma and other predominantly pediatric supratentorial papillary/epithelioid gliomas

Astroblastomas are rare primary brain tumors with no currently assigned World Health Organization grade. In their classical presentation, they are well-circumscribed supratentorial solid-cystic tumors with minimal peritumoral T2 hyperintensity. They can be difficult to distinguish from other predominantly pediatric supratentorial papillary/epithelioid gliomas, such as ependymomas, choroid plexus tumors, papillary glioneuronal tumors, papillary tumors of the pineal region, and occasionally atypical teratoid/rhabdoid tumor of the central nervous system. Distinct morphologic, immunohistochemical, and ultrastructural features aid in reaching a definitive diagnosis. In this review, we present a case of pediatric astroblastoma with a unique course and discuss the key differences and similarities between astroblastomas and other predominantly pediatric supratentorial papillary/epithelioid gliomas.