Review of the molecular genetics and chemotherapeutic treatment of adult and paediatric medulloblastoma.
Medulloblastoma is the most common primary brain tumour in children and accounts for 25% of newly diagnosed cases. Recent advances in treatment have extended 5-year survival rates from 3 - > 70% during the past 50 years. These improvements in survival have resulted from a multi-modality approach that includes surgical resection, posterior fossa and craniospinal irradiation and chemotherapy for selected, high-risk patients. The literature regarding chemotherapy of adult and paediatric patients is reviewed in-depth. The most active agents include cisplatin, CCNU, cyclophosphamide, vincristine and carboplatin. Although patients are living longer with their disease, neurocognitive function and quality of life are often impaired following radiation therapy (RT) to the developing brain. To safely allow reductions in the dose of RT, the specificity and efficacy of chemotherapy must be improved. Recent advances in the molecular genetics of medulloblastoma transformation (e.g., myc, PTCH ) are reviewed and discussed. A thorough understanding of these pathways will be critical for the development of more specific, novel drugs. Further clinical trials will be needed to evaluate the activity of these new drugs and determine their role in the treatment plan of patients with medulloblastoma.
Newton
H B
HB
Department of Neurology, The Ohio State University Hospitals, 465 Means Hall, 1654 Upham Drive, Columbus, Ohio 43210, USA. newton.12@osu.edu
D000293:Adolescent; D000328:Adult; D000368:Aged; D000818:Animals; D000970:Antineoplastic Agents / Q000494:pharmacology / Q000627:therapeutic use*; D016026:Bone Marrow Transplantation; D001932:Brain Neoplasms / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D008527:Medulloblastoma / Q000188:drug therapy* / Q000235:genetics* / Q000473:pathology / Q000628:therapy; D008875:Middle Aged; D008967:Molecular Biology
D000970:Antineoplastic Agents