Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling

authors

• Figarella-Branger Dominique B
• Le Lechapt-Zalcman E
• Tabouret E T
• Jünger Stephanie
• Maues de Paula André
• Bouvier Corinne
• Colin Carole
• Jouvet Anne
• Forest Fabien F
• Andreiuolo Felipe
• Quintin-Roue Isabelle
• Machet Marie-Christine
• Heitzmann Anne
• Milin Serge
• Sevestre Henri
• Godfraind Catherine
• Labrousse François F
• Metellus Philippe
• Scavarda Didier
• Pietsch Torsten T

keywords

• Trisomy 19
• Intracranial
• Clear cell ependymomas
• RelA
• NF kappa B

document type

abstract

Clear cell ependymoma is one of the 4 main histological subtypes of ependymomas defined by the World Health Organization (WHO) classification of tumors of the CNS. DNA methylation profiling can distinguish 4 subgroups of intracranial ependymomas, including supratentorial (ST) ependymomas with Yes-associated protein 1 fusion (YAP1), ST ependymomas with fusion of v-rel avian reticuloendotheliosis viral oncogene homolog A (RELA), posterior fossa ependymomas with balanced genome, and posterior fossa ependymomas with chromosomal instability. In addition, trisomy 19 is a genomic hallmark of ependymomas with rich branching capillaries. However, the relation of histological and molecular subtypes is unclear.

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