Medulloblastoma, primitive neuroectodermal tumors, and ependymoma

Medulloblastoma, primitive neuroectodermal tumor (PNET), and ependymoma are the most frequent malignant brain tumors of childhood. Since the 1980s, multimodal therapy optimization trials of the German Society of Pediatric Oncology and Hematology (GPOH) have led to improved survival rates. Within the current primary treatment trial HIT 2000, modern strategies for radiotherapy and chemotherapy are evaluated according to histology, staging, and age after maximal safe tumor resection. Young children with desmoplastic medulloblastoma receive chemotherapy alone, and the aim for young children with classic medulloblastoma and PNET is for chemotherapy to delay radiotherapy. Intensified treatment concepts are evaluated in metastatic disease. In ependymoma, the extent of resection and local radiotherapy are most important. Prolonging survival while preserving a good quality of life is the aim of the trial HIT-REZ 2005 for children with relapsed medulloblastoma, PNET, or ependymoma; it includes the evaluation of new drugs and treatment modalities (temozolomide, intraventricular etoposide). Central tumor banking and improved treatment stratification by prognostic molecular markers are planned for future trials.