Epithelioid glioblastomas (eGBMs) are among the rarest variants of glioblastoma with an overrepresentation of BRAF V600E mutations in 50% or more cases.¹ 

There are three diagnostic subsets based on molecular analysis ¹:

1. PXA-like most commonly in children and young adults
2. IDH-wild type GBM-like most commonly in older adults, and
3. RTK1 GBM-like most commonly in children and young adults

Pediatric epithelioid glioblastomas may present with acute hemorrhage. Leptomeningeal dissemination and metastases outside the CNS are possible.² 

As with most glioblastomas, eGBMs are best followed with MRI. Radiographic features found in the majority of tumors include diffusion restriction of the solid tumor component and perilesional vasogenic edema. Central necrosis may also be present, with the degree of necrosis being a negative prognostic factor. Mass effect may also be present depending on the size of the tumor and the extent of edema. These tumors are most frequently located in the cerebrum and diencephalon.³

This patient presented as an adolescent with a 1-month history of memory loss and aphasia. She subsequently had a resection with adjuvant chemotherapy and radiation treatment. 

Case authors: Le J, Senti M, Moore A