Article

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. 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 compromize outcome. CONCLUSION:
Management of patients with medulloblastoma implies a multidisciplinary approach
combining the contributions of neurosurgery, neuroradiology, pediatric oncology,
neuro-oncology and radiotherapy teams.
CI - Copyright (c) 2011 Elsevier Masson SAS. All rights reserved.

Langue : FRANCAIS