[Medulloblastomas: review].
Fiche publication
Date publication
mai 2011
Auteurs
Membres identifiés du Cancéropôle Est :
Dr BERNIER-CHASTAGNER Valérie, Pr CHASTAGNER Pascal, Pr TAILLANDIER Luc
Tous les auteurs :
Taillandier L, Blonski M, Carrie C, Bernier V, Bonnetain F, Bourdeaut F, Thomas IC, Chastagner P, Dhermain F, Doz F, Frappaz D, Grill J, Guillevin R, Idbaih A, Jouvet A, Kerr C, Donadey FL, Padovani L, Pallud J, Sunyach MP
Lien Pubmed
Résumé
INTRODUCTION: The 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 KNOWLEDGE: Diagnosis 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 compromise outcome. PERSPECTIVES AND CONCLUSION: Management of patients with medulloblastoma implies a multidisciplinary approach combining the contributions of neurosurgery, neuroradiology, pediatric oncology, neuro-oncology and radiotherapy teams.
Référence
Rev Neurol (Paris). 2011 May;167(5):431-48