Toward an integrated histomolecular diagnosis of supratentorial ependymoma

In an editorial in the July 2016 issue of Neuro-Oncology, Dr. Kristian W. Pajtler -- from the Department of Pediatric Oncology at the University Hospital, Heidelberg, Germany -- reflects on the fact that ependymomas are no longer considered a single disease entity regardless of location or molecular profile. Three ependymoma subgroups have now been identified in the supratentorial (ST) compartment. Pajtler writes: "One subgroup was enriched for World Health Organization (WHO) grade I tumors, and termed “molecular” subependymoma (ST-SE), while the other 2 were characterized by mutually exclusive prototypic fusion genes involving YAP1 and RELA, and named ST-EPN-YAP1 and ST-EPN-RELA, respectively." The RELA fusion ependymoma is notable because it is recognized as a separate diagnostic entity in the latest WHO classification of CNS tumors. Pajtler goes on as follows: "The study by Figarella-Branger et al. published in this issue of Neuro-Oncology focuses on supratentorial clear cell ependymoma... The authors report on a rare series of 20 supratentorial clear cell ependymomas that all share an additional histopathological attribute, the appearance of branching capillaries... Interestingly, nuclear immunostaining with a p65/RELA antibody was detected in all cases. The authors identified a causative C11orf95-RELA fusion using reverse transcriptase (RT)-PCR primers for the most common subtype, RELA fusion type I, in 19 of 20 samples... Based on these findings, the authors conclud...
Source: neuropathology blog - Category: Radiology Tags: neoplasms Source Type: blogs