Treatment strategies for medulloblastoma and primitive neuroectodermal tumors

Medulloblastoma and primitive neuroectodermal tumors (PNETs) are the most common malignant brain tumors in children. The concern for late sequelae of neuraxis irradiation and the obligation to improve disease-free survival in children who harbor malignant brain tumors has led to the additional provision of systemic chemotherapy to standard- and reduced-dose radiotherapy, as well as to the evaluation of alternate modes of radiotherapy delivery. Analysis of evidence has suggested that chemotherapy has an impact on length of survival in children with medulloblastoma and PNETs. The question remains as to whether chemotherapy combined with reduced-dose radiotherapy provides greater benefit than standard-dose radiotherapy alone, and which subset of children the treatment most benefits. Also unanswered is the question of whether chemotherapy can serve as the primary treatment in infants with these lesions. In an attempt to help answer these questions, the authors review the major chemotherapy and radiotherapy trials for newly diagnosed patients and those with recurrent medulloblastoma and PNETs.