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

authors

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

keywords

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

document type

ART

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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