title: Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling.
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.
  
  
  
  Here, we report a series of 20 ependymomas histologically defined by clear cells and branching capillaries.
  
  
  
  We observed a strong male predominance. Median age at surgery was 10.4 years (range, 0.8-68.4). All cases were ST, cortical, contrast enhancing, and most often frontal, cystic, and calcified. All tumors qualified as WHO grade III. Some of them exhibited neuronal differentiation. Trisomy 19 was recorded in 13 cases. All samples strongly accumulated p65RelA protein within nuclei, indicating pathological activation of the nuclear factor-kappaB pathway. We identified causative C11ORF95-RELA fusion in almost all cases. Median progression-free survival and overall survival were 11.4 years (95% CI: 5.1-17.8) and not reached, respectively.
  
  
  
  ST clear cell ependymomas with branching capillaries display characteristic clinicopathological features and are associated with pathological activation of nuclear factor-kappaB signaling, which may indicate a potential novel target for therapy in these patients.
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Dominique
authors.initials: D
authors.lastname: Figarella-Branger
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Emmanuèle
authors.initials: E
authors.lastname: Lechapt-Zalcman
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Emeline
authors.initials: E
authors.lastname: Tabouret
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Stephanie
authors.initials: S
authors.lastname: Jünger
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: André Maues
authors.initials: AM
authors.lastname: de Paula
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Corinne
authors.initials: C
authors.lastname: Bouvier
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Carole
authors.initials: C
authors.lastname: Colin
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Anne
authors.initials: A
authors.lastname: Jouvet
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Fabien
authors.initials: F
authors.lastname: Forest
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Felipe
authors.initials: F
authors.lastname: Andreiuolo
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Isabelle
authors.initials: I
authors.lastname: Quintin-Roue
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Marie-Christine
authors.initials: MC
authors.lastname: Machet
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Anne
authors.initials: A
authors.lastname: Heitzmann
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Serge
authors.initials: S
authors.lastname: Milin
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Henri
authors.initials: H
authors.lastname: Sevestre
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Catherine
authors.initials: C
authors.lastname: Godfraind
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: François
authors.initials: F
authors.lastname: Labrousse
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Philippe
authors.initials: P
authors.lastname: Metellus
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Didier
authors.initials: D
authors.lastname: Scavarda
authors.affiliation: Department of Pathology and Neuropathology, Assistance Publique des Hôpitaux de Marseille (APHM), CHU Timone, Marseille, France (D.F.-B, E.T., A.M.d.P, C.B.); Aix-Marseille Université, Inserm, Marseille, France (D.F.-B, E.T., C.B., C.C.); Department of Pathology, CHU Caen, Hôpital de la Côte de Nacre, Caen, France (E.L.-Z); Department of Neurooncology, APHM, Marseille, France (E.T.); Department of Neuropathology, University of Bonn, Medical Center Sigmund-Freud, Bonn, Germany (S.J., T.P.); Department of Pathology and Neuropathology, Hospices civils de Lyon, Bron, France (A.J.); Department of Pathology, CHU de Saint-Etienne, Saint-Etienne, France (F.F.); Department of Neuropathology, CHU Sainte Anne, Paris, France (F.A.); Department of Pathology, CHU de Brest, Hôpital de la Cavale Blanche, Brest, France (I.Q.-R); Department of Pathology, CHU de Tours, Hôpital Trousseau, Tours, France (M.-C.M.); Department of Pathology, CHR d'Orléans, Hôpital de la Source, Orléans, France (A.H.); Department of Pathology, CHU de Poitiers, Hôpital la Milétrie, Poitiers, France (S.M.); Department of Pathology, CHU d'Amiens, Amiens, France (H.S.); Department of Pathology, CHU de Clermont-Ferrand, Clermont-Ferrand, France (C.G.); Department of Pathology, CHU de Limoges, Limoges, France (F.L.); Department of Neurosurgery, APHM, CHU Timone, Marseille, France (P.M.); Department of Pediatric Neurosurgery, APHM, CHU Timone, Marseille, France (D.S.).
authors.forename: Torsten
authors.initials: T
authors.lastname: Pietsch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000378:metabolism* / Q000401:mortality / Q000473:pathology*; D002196:Capillaries / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004806:Ependymoma / Q000378:metabolism* / Q000401:mortality; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D016328:NF-kappa B / Q000378:metabolism*; D015398:Signal Transduction / Q000235:genetics; D055815:Young Adult
chemical_list: D016328:NF-kappa B
keywords: NF kappa B; RelA; clear cell ependymomas; intracranial; trisomy 19