Meryl Horwitz1, Christelle Dufour2, Pierre Leblond3, Franck Bourdeaut4,5, Cécile Faure-Conter6, Anne-Isabelle Bertozzi7, Marie Bernadette Delisle8, Gilles Palenzuela9, Anne Jouvet10, Didier Scavarda11, Matthieu Vinchon12, Laetitia Padovani13, Jean Gaudart14,15, Dominique Figarella Branger16, Nicolas Andre17,18,19. 1. Department of Pediatric Hematology-Oncology, APHM, La Timone Hospital, 264 rue Saint-Pierre, 13005, Marseille, France. meryl.horwitz@gmail.com. 2. Department of Pediatric and Adolescent Oncology, Gustave Roussy Campus, 114, rue Edouard-Vaillant, 94805, Villejuif Cedex, France. christelle.dufour@gustaveroussy.fr. 3. Department of Pediatric Oncology, Oscar Lambret, 3 rue Frederic Combemale, BP 307, 59020, Lille Cedex, France. p-leblond@o-lambret.fr. 4. Department of Pediatric Oncology, Institut Curie, 26, rue d'Ulm, 75005, Paris, France. franck.bourdeaut@curie.fr. 5. INSERM U830, Laboratory of Genetics and Cancer Biology, Institut Curie, Paris, France. franck.bourdeaut@curie.fr. 6. Department of Pediatrics, Centre Léon Bérard, 28 Prom. Léa et Napoléon Bullukian, 69008, Lyon, France. cecile.conter@ihope.fr. 7. Department of Hematology-Oncology, Children University Hospital, 330 Avenue de Grande Bretagne, 31300, Toulouse, France. bertozzi.ai@chu-toulouse.fr. 8. Department of Neuropathology, Toulouse Rangueil University Hospital, 1, avenue du Pr Jean Poulhès, 31400, Toulouse, France. delisle.b@chu-toulouse.fr. 9. Department of Pediatric Hematology-Oncology, University Hospital of Montpellier, 191, avenue du doyen Gaston Giraud, 34295, Montpellier cedex, France. gillespalen@yahoo.fr. 10. Department of Pathology and neuropathology, "Pierre Werthmeier" Hospital, Boulevard Pinel, 69677, Bron, France. anne.jouvet@chu-lyon.fr. 11. Department of Neurosurgery, APHM, La Timone Hospital, 264 rue Saint-Pierre, 13005, Marseille, France. didier.scavarda@ap-hm.fr. 12. Department of Pediatric Neurosurgery, Lille University Hospital, 2 Avenue Oscar Lambret, 59000, Lille, France. matthieu.vinchon@chru-lille.fr. 13. Department of Radiation Oncology and Pediatrics, APHM, La Timone Hospital, 264 rue Saint-Pierre, 13005, Marseille, France. laetitia.padovani@ap-hm.fr. 14. BIOSTIC, Pôle de Santé Publique, AP-HM, Marseille, 264 rue Saint-Pierre, 13005, Marseille, France. jean.gaudart@ap-hm.fr. 15. UMR912 SESSTIM, Aix-Marseille University, Marseille, France. jean.gaudart@ap-hm.fr. 16. Department of Pathology, APHM, La Timone Hospital, Université Aix-Marseille, 264 rue Saint-Pierre, 13005, Marseille, France. dominiqueFrance.figarella@ap-hm.fr. 17. Department of Pediatric Hematology-Oncology, APHM, La Timone Hospital, 264 rue Saint-Pierre, 13005, Marseille, France. nicolas.andre@ap-hm.fr. 18. INSERM UMR 911, Centre de Recherche en Oncologie biologique et en Oncopharmacologie, Aix-Marseille University, 264 rue Saint-Pierre, 13005, Marseille, France. nicolas.andre@ap-hm.fr. 19. Department of Pediatric Hematology and Oncology, Children Hospital of La Timone, 264, rue Saint-Pierre, 13005, Marseille, France. nicolas.andre@ap-hm.fr.
Abstract
PURPOSES: The purpose of this study was to retrospectively study embryonal tumors with multilayered rosettes (ETMR), a rare new entity that gathers ETAN-TR (embryonal tumor with abundant neuropil and true rosettes), ependymoblastomas, and medulloepitheliomas, in order to improve their descriptions and try to better define therapeutic modalities. METHODS: Patients with ETMR, ETAN-TR, ependymoblastoma, and medulloepithelioma treated in SFCE centres (Société Française de lutte contre les Cancers et les leucémies de l'Enfant et de l'adolescent) since 2000 were collected. Data were retrieved from clinical charts. RESULTS: Thirty-eight patients were included in the analysis. Seventeen had an ETAN-TR, 13 had a medulloepithelioma, and 8 had an ETMR. No ependymoblastoma was included. The median age at diagnosis was 31 months (range, 2.8-141 months). The predominant tumor location was supratentorial (66%); 18.4% patients had metastatic lesion. LIN28A expression was positive in 11/11 patients. Amplification of the locus 19q13.42 was positive in 10/12 patients. Thirty patients were treated according to the primitive neuroectodermal tumors of high risk (PNET-HR) protocol. The median time of follow-up was 0.9 years (range 0.1 to 15.3 years). The 1-year event-free survival (EFS) and overall survival (OS) were, respectively, 36% CI 95% (23-55) and 45% CI 95% (31-64). On multivariate analysis, complete surgical resection, radiotherapy, and high-dose chemotherapy were associated with a better overall survival with a relative risk of, respectively, 7.9 CI 95% (2.6-23.5) p < 0.0002, 41.8 CI 95% (9.4-186) p < 0.0001, and 3.5 CI 95% (1.3-9.5) p = 0.012. CONCLUSION: Prognosis of ETMR remains dismal despite multimodal therapy. LIN28A immunostaining and 19q13.42 amplification should be systematically done to secure the diagnosis. Complete surgical resection, radiotherapy, and high-dose chemotherapy are associated with better outcome.
PURPOSES: The purpose of this study was to retrospectively study embryonal tumors with multilayered rosettes (ETMR), a rare new entity that gathers ETAN-TR (embryonal tumor with abundant neuropil and true rosettes), ependymoblastomas, and medulloepitheliomas, in order to improve their descriptions and try to better define therapeutic modalities. METHODS:Patients with ETMR, ETAN-TR, ependymoblastoma, and medulloepithelioma treated in SFCE centres (Société Française de lutte contre les Cancers et les leucémies de l'Enfant et de l'adolescent) since 2000 were collected. Data were retrieved from clinical charts. RESULTS: Thirty-eight patients were included in the analysis. Seventeen had an ETAN-TR, 13 had a medulloepithelioma, and 8 had an ETMR. No ependymoblastoma was included. The median age at diagnosis was 31 months (range, 2.8-141 months). The predominant tumor location was supratentorial (66%); 18.4% patients had metastatic lesion. LIN28A expression was positive in 11/11 patients. Amplification of the locus 19q13.42 was positive in 10/12 patients. Thirty patients were treated according to the primitive neuroectodermal tumors of high risk (PNET-HR) protocol. The median time of follow-up was 0.9 years (range 0.1 to 15.3 years). The 1-year event-free survival (EFS) and overall survival (OS) were, respectively, 36% CI 95% (23-55) and 45% CI 95% (31-64). On multivariate analysis, complete surgical resection, radiotherapy, and high-dose chemotherapy were associated with a better overall survival with a relative risk of, respectively, 7.9 CI 95% (2.6-23.5) p < 0.0002, 41.8 CI 95% (9.4-186) p < 0.0001, and 3.5 CI 95% (1.3-9.5) p = 0.012. CONCLUSION: Prognosis of ETMR remains dismal despite multimodal therapy. LIN28A immunostaining and 19q13.42 amplification should be systematically done to secure the diagnosis. Complete surgical resection, radiotherapy, and high-dose chemotherapy are associated with better outcome.
Entities:
Keywords:
Brain tumors; Chemotherapy; Children; ETMR; Radiotherapy; Surgery
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