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

Dominique B Figarella-Branger (1, 2) , E Le Lechapt-Zalcman (3) , E T Tabouret (4, 1, 2) , Stephanie Jünger (5) , André Maues de Paula (1) , Corinne Bouvier (1, 2) , Carole Colin (2) , Anne Jouvet (6) , Fabien F Forest (7) , Felipe Andreiuolo (8) , Isabelle Quintin-Roue (9) , Marie-Christine Machet (10) , Anne Heitzmann (11) , Serge Milin (12) , Henri Sevestre (13) , Catherine Godfraind (14) , François F Labrousse (15) , Philippe Metellus (16) , Didier Scavarda (17) , Torsten T Pietsch (17)

1 ACPNP - Service d'Anatomo-Cyto-Pathologie et de NeuroPathologie [Hôpital de la Timone - APHM]
2 CRO2 - Centre de Recherches en Oncologie biologique et Oncopharmacologie
3 Département de Pathologie [CHU Caen]
4 Neuro-Oncologie [Hôpital de la Timone - APHM]
5 University of Bonn
6 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
7 CHU de Saint-Etienne - Centre Hospitalier Universitaire de Saint-Etienne
8 Centre Hospitalier Sainte Anne [Paris]
9 CHU - AnaPath - CHRU Brest - Laboratoire d'Anatomo-Pathologie
10 Service de pathologie [CHU Trousseau]
11 CHRO - Centre Hospitalier Régional d'Orléans
12 CHU Poitiers - Centre hospitalier universitaire de Poitiers
13 CHU Amiens-Picardie
14 CHU Clermont-Ferrand
15 Service d'Anatomie Pathologique [CHU Limoges]
16 Service de neurochirurgie
17 Pédiatrie et oncologie pédiatrique [Hôpital de la Timone - APHM]

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.

Keywords: trisomy 19; intracranial; clear cell ependymomas; RelA; NF kappa B

Domains: Life Sciences [q-bio]; Neurons and Cognition [q-bio.NC]; Neurobiology; Life Sciences [q-bio]; Human health and pathology; Tissues and Organs [q-bio.TO]; Life Sciences [q-bio]; Cancer

Dominique B Figarella-Branger, E Le Lechapt-Zalcman, E T Tabouret, Stephanie Jünger, André Maues de Paula, et al.. Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling. Neuro-Oncology, 2016, 18, pp.919-927. ⟨10.1093/neuonc/now025⟩. ⟨hal-01478840⟩