Childhood medulloblastoma: current status of biology and treatment

Abstract

Medulloblastoma, a primitive neuro-ectodermal tumour that arises in the posterior fossa, is the most common malignant brain tumour occurring in childhood. Over the past half century, the long-term survival for children with medulloblastoma has improved remarkably from a certain fatal diagnosis to a cancer that is often curable. Although overall survival for children with non-disseminated and non-anaplastic medulloblastoma can approach 80%, the current multidisciplinary therapeutic approach is not without long-term sequelae. Chemotherapy has improved the long-term survival and allowed for reductions in the amount of radiation given, thereby reducing some of the long-term toxicities. In this review, we describe the current understanding of the basic biology of medulloblastoma and report on the current active chemotherapeutic agents utilized in medulloblastoma therapy. Ultimately, our understanding of the basic biology of medulloblastoma may lead to further advances in therapy by providing targets that are more specific and potentially less toxic.