Childhood medulloblastoma: progress and future challenges

Abstract

Medulloblastoma and other primitive neuroectodermal tumors are the most common malignant tumors of childhood. Progress has been slowly made in the management of such tumors. Long-term neurocognitive sequelae of treatment in children with medulloblastoma are common and recent treatment trials have attempted to reduce the amount of craniospinal radiation therapy or delay radiation therapy in attempts to reduce such sequelae. Surgery remains a critical component of treatment, although there is increasing concern about surgically-related complications, including the cerebellar mutism syndrome. For older children, craniospinal radiation remains an integral part of management. However, recent studies have suggested an excellent outcome after reduced-dose craniospinal radiation therapy and adjuvant chemotherapy. The role of chemotherapy is expanding for children with medulloblastoma and is now presently a component of protocols for children with average-risk and poor-risk disease. For children with poor-risk disease, intensifications of chemotherapy both during and after radiation therapy are presently being explored. Treatment of infants and young children with medulloblastoma remains problematic and a variety of different approaches are being investigated with the aims of both improving outcome and reducing long-term sequelae.