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Medulloblastomas: review |
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| Authors: | Taillandier L Blonski M Carrie C Bernier V Bonnetain F Bourdeaut F Thomas I-C Chastagner P Dhermain F Doz F Frappaz D Grill J Guillevin R Idbaih A Jouvet A Kerr C Donadey F-L Padovani L Pallud J Sunyach M-P |
| Institution: | a Unité de neuro-oncologie, service de neurologie, CHU hôpital central, 29, avenue de-Lattre-de-Tassigny, 54035 Nancy cedex, France b Département de radiothérapie, centre Léon-Bérard, 28, rue Laennec, 69373 Lyon, France c Département de radiothérapie, centre A-Vautrin, 54500 Vandoeuvre-lès-Nancy, France d Unité de biostatistique centre GF Leclerc, 1, rue du Professeur Marion, BP 77980, 21079 Dijon cedex, France e Centre de recherche et unité de génétique somatique, institut Curie, 22, rue d’Ulm, 75231 Paris, France f Service d’oncologie, hôpital Saint-André, 1, rue Jean-Burguet, 33075 Bordeaux, France g Service d’oncologie pédiatrique, hôpital d’enfant, CHU de Nancy, Brabois, 54500 Vandoeuvre-lès-Nancy, France h Département de radiothérapie, institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif, France i Service d’oncologie pédiatrique, institut Curie, 26, rue d’Ulm, 75231 Paris, France j Oncologie, centre Léon-Bérard, 28, rue Laennec, 69373 Lyon, France k Oncologie pédiatrique, institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif, France l Département de neuroradiologie, hôpital de la Salpêtrière, 47, boulevard de l’hôpital, 75013 Paris, France m Inserm U711 et neurologie Mazarin, hôpital de la Salpêtrière, 47, boulevard de l’hôpital, 75013 Paris, France n Service d’anatomopathologie, hôpital neurologique, 59, boulevard de l’hôpital, 69394 Lyon, France o Département de radiothérapie, centre Val-d’Aurelle, 208, rue des Apothicaires, 34298 Montpellier, France p Département de radiothérapie, hôpital de la Timone, 13385 Marseille, France q Service de neurochirurgie, faculté Paris-Descartes, centre hospitalier Sainte-Anne, 1, rue Cabanis, 75674 Paris cedex 14, France |
| Abstract: | IntroductionThe term of “medulloblastoma” refers to cerebellar tumors belonging to the family of primitive neuro-ectodermic tumors (PNET). Medulloblastomas represent 40% of cerebellar tumors, 15 to 20% of brain tumors and the first cause of malignant brain tumors in childhood. Seventy to 80% of cases are diagnosed in children versus 20 to 30% in adults.Updated knowledgeDiagnosis is based on clinical and radiological exams, and proved on pathological analysis in association with molecular biology. Treatment comprises surgery, craniospinal radiotherapy except for children under five years of age and chemotherapy according to age and high-risk criteria. Medulloblastoma is a rare case of a central nervous system tumor which is radio- and chemo-sensitive. Treatment goals are, on one hand, to improve the survival rates and, on the other hand, to avoid late neurocognitive, neuroendocrine and orthopedic side effects related to radiation therapy, notably in children. The prognosis is relatively good, with a five year survival rate over 75% after complete resection of a localized tumor although sequelae may still compromize outcome.Perspectives and conclusionManagement of patients with medulloblastoma implies a multidisciplinary approach combining the contributions of neurosurgery, neuroradiology, pediatric oncology, neuro-oncology and radiotherapy teams. |
| Keywords: | Medulloblastome Chirurgie Radiothé rapie Chimiothé rapie Cognition Qualité de vie |
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