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This document, published in Acta Neuropathologica Communications, discusses the identification of a subtype of diffuse midline glioma (DMG) called H3K27/MAPKinase co-altered. The study analyzes a cohort of 182 pediatric and adult cases of DMG, H3K27-altered and identifies 30 tumors with a loss of ATRX expression. The authors provide clinical, histopathological, and molecular data on these tumors and suggest that the loss of ATRX expression may be a potential diagnostic tool for identifying DMG, H3K27/MAPK co-altered. The study highlights the distinct features of this subtype and emphasizes the importance of integrating clinical, radiological, histopathological, and molecular results for an accurate diagnosis. [Extracted from the article]

According to our results, it may be recommended to perform L1CAM and NF B immunohistochemistry and to search for clinical and radiological criteria for NF2 before performing DNA-methylation profiling for the differential diagnosis of pediatric PFB ependymoma. 1) segregated tumors into: 10 PFB (66.7%, with an enrichment of subclass 4 as previously reported [[1]]), two ependymomas, I ZFTA- i fusion positive (13.3%), and two NF2-associated spinal ependymomas (13.3%). Radiological and histomolecular features of reclassified posterior fossa ependymomas as spinal ependymomas Case 12 (A - E): A - C IRM in a NF2 patient showing bilateral vestibular schwannomas, multiple meningiomas (B, C) and a median intra-parenchymal mass with high contrast enhancement in the bulbo-medullary junction. [Extracted from the article]