ArticleNeuro-oncology2016
Supratentorial clear cell ependymomas with branching capillaries demonstrate characteristic clinicopathological features and pathological activation of nuclear factor-kappaB signaling.
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In one paragraph
Article in Neuro-oncology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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43citing papers in PubMed
7.3field-weighted citation impact, top 2% of its field
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
43 citing papers in PubMed, 106 citations in OpenAlex.
- AOSNP-ADAPTR resource level-based recommendations on practical diagnostic strategies for ependymomas.Brain pathology (Zurich, Switzerland) · 2026Article
- A brief history of ependymoma.Neuro-oncology · 2026Review
- TRAF7-Mutated Fibromyxoid Spindle Cell Tumor of Bone: An Osseous Case Expanding the Spectrum of TRAF7-Mutated Tumors With Over 20 Years Clinical Follow-Up.Genes, chromosomes & cancer · 2026Article
- Supra-tentorial Ependymomas with ZFTA Fusion, YAP1 Fusion, and Astroblastomas, MN1-altered: Characteristic Imaging Features.Clinical neuroradiology · 2024Article
- Ependymoma from Benign to Highly Aggressive Diseases: A Review.Advances and technical standards in neurosurgery · 2024Review
- Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?Frontiers in molecular neuroscience · 2024Review
- Review and update on pediatric ependymoma.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Review
- A clinicopathological analysis of supratentorial ependymoma, ZFTA fusion-positive: utility of immunohistochemical detection of CDKN2A alterations and characteristics of the immune microenvironment.Brain tumor pathology · 2023Article
- Changes to pediatric brain tumors in 2021 World Health Organization classification of tumors of the central nervous system.Pediatric radiology · 2023Review
- Adenomatoid tumors of ovary mimicking malignancy: report of 2 cases and literature review.BMC women's health · 2022Review
- Diffuse GFAP Immunopositivity in the Oligodendrocyte-like Component of Pilocytic Astrocytoma Distinguishes It from Mimickers.Diagnostics (Basel, Switzerland) · 2022Article
- The biology of ependymomas and emerging novel therapies.Nature reviews. Cancer · 2022Review
- Supratentorial ependymoma, zinc finger translocation-associated fusion positive, with extensive synaptophysin immunoreactivity arising from malignant transformation of clear cell ependymoma: A case report.Surgical neurology international · 2022Article
- Supratentorial non-RELA, ZFTA-fused ependymomas: a comprehensive phenotype genotype correlation highlighting the number of zinc fingers in ZFTA-NCOA1/2 fusions.Acta neuropathologica communications · 2021Article
- Pediatric ependymoma: an overview of a complex disease.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2021Review
- A coordinated approach for the assessment of molecular subgroups in pediatric ependymomas using low-cost methods.Journal of molecular medicine (Berlin, Germany) · 2021Article
- Review
- Supratentorial pediatric cortical ependymomas: a comprehensive retrospective study.Neurosurgical review · 2021Review
- Ependymoma-like tumor with mesenchymal differentiation harboring C11orf95-NCOA1/2 or -RELA fusion: A hitherto unclassified tumor related to ependymoma.Brain pathology (Zurich, Switzerland) · 2021Article
- Supratentorial ependymoma in childhood: more than just RELA or YAP.Acta neuropathologica · 2021Article
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Authors and funding
20 authors at 7 institutions in 2 countries.
Dominique Figarella-BrangerDepartment 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.).
Emmanuèle Lechapt-ZalcmanDepartment 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.).
Emeline TabouretDepartment 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.).
Stephanie JüngerDepartment 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.).
André Maues de PaulaDepartment 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.).
Corinne BouvierDepartment 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.).
Carole ColinDepartment 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.).
Anne JouvetDepartment 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.).
Fabien ForestDepartment 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.).
Felipe AndreiuoloDepartment 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.).
Isabelle Quintin-RoueDepartment 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.).
Marie-Christine MachetDepartment 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.).
Anne HeitzmannDepartment 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.).
Serge MilinDepartment 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.).
Henri SevestreDepartment 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.).
Catherine GodfraindDepartment 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.).
François LabrousseDepartment 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.).
Philippe MetellusDepartment 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.).
Didier ScavardaDepartment 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.).
Torsten PietschDepartment 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.).
Hôpital de la Timone · FRCentre Hospitalier Universitaire de Caen · FRUniversité de Caen Normandie · FRInserm · FRCentre Hospitalier Universitaire de Tours · FRAssistance Publique Hôpitaux de Marseille · FRHôpital Armand-Trousseau · FR
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundClear 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.
methodsHere, we report a series of 20 ependymomas histologically defined by clear cells and branching capillaries.
resultsWe 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.
conclusionST 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.
Indexed as
Signal TransductionAdolescentAdultAgedBrain NeoplasmsCapillariesChildChild, PreschoolDisease-Free SurvivalEpendymomaHumansInfantMaleMiddle AgedNF-kappa BYoung AdultNF-kappa Bclear cell ependymomasintracranialNF kappa BRelAtrisomy 19
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