Diffuse intrinsic pontine glioma-like tumor with EZHIP expression and molecular features of PFA ependymoma.

Diffuse brainstem gliomas, historically termed diffuse intrinsic pontine glioma (DIPG), account for approximately 75% of pediatric brainstem tumors and have a particularly poor prognosis with a median survival of only 10 months [[8], [10]]. To further assess the methylation profile of this tumor in relation to other CNS entities, we performed unsupervised clustering on the DKFZ cohort that comprises the 82 tumor methylation classes used in the Classifier (v11b4). The "EPN, PF A" tumor class contains tumors arising solely within the fourth ventricle and/or cerebellum. EZHIP (CXorf67) immunohistochemistry showed increased nuclear expression in tumor cells (d) and served to highlight individual tumor cells percolating surrounding normal-appearing brain (e-f). [Extracted from the article]