Medulloblastoma

Abstract

Medulloblastoma is a highly malignant small round blue cell tumor of the posterior fossa. It accounts for approximately 20% of all brain tumors in children, is the most common malignant brain tumor of childhood and, with greater advances against other common cancers in children, such as acute leukemias, is currently responsible for about 10% of all childhood cancer deaths. Around 30% of patients present with metastatic disease, which is essentially restricted to the central nervous system. Since the 1970s the application of multimodal therapy including maximal resection followed by craniospinal irradiation and chemotherapy have transformed a disease that was invariably fatal when treated with surgery alone to one which can be cured for approximately 70% of all patients. The advent genome revolution has seen uncovered the four core molecular subgroups of medulloblastoma, each with distinct clinical behavior and survival outcomes. Future medulloblastoma clinical trials will see the adoption of an integrated classification system based on clinical, histological and molecular criteria in order to allow more precisely risk-stratified therapy. This will permit the incorporation of medulloblastoma subgroup specific therapies. This chapter comprehensively covers the field of medulloblastoma, from basic histology and radiology to clinical trials conducted over the past three decades, to the more recently identified molecular subgroups.