Actual and future strategies in interdisciplinary treatment of medulloblastomas, supratentorial PNET and intracranial germ cell tumors in childhood

Background: The chances for cure in medulloblastoma, supratentorial primiti ve neuroectodermal tumors (stPNET) and intracranial germ cell tumors have d ecisively improved within the Last decades. Today Long-term survival in the range between 60% and 80% and more than 90%, respectively, can be achieved . The Low incidence of brain tumors in childhood and the necessity for opti mal patient care has Led to the fact that more than 90% of children are tre ated within national and international controlled studies today in order to assure a constant improvement of therapeutic outcome. Recent developments in neurosurgery achieved complete tumor resections in the majority of child ren at a low risk for morbidity and mortality. Methods: Systemic irradiation of neuroaxis is an essential part in the mana gement of medulloblastoma, stPNET and intracranial germ cell tumors. The in troduction of quality assurance programs in radiooncology assures a precise radiotherapy of target volumes and is a prerequisite to improve survival. Results: Hyperfractionated radiotherapy has the potential of increasing dos e to tumor more safely without increasing the risk for Late adverse effects . Pilot studies revealed excellent tumor control in medulloblastoma with ac ceptable acute toxicity and a Longterm survival of up to 96%. In medullobla stoma stereotactic radiation techniques reveal an acceptable toxicity and p romising results in tumor control in recurrent disease or as primary treatm ent. They are now part of future treatment protocols in case of persisting residual tumor. Radiotherapy alone in pure germinoma is continuously yieldi ng high cure rates. In secreting germ cell tumors cisplatin containing chem otherapies in conjunction with radiotherapy achieve a Long-term survival ra te of 80% today. Especially in high risk medulloblastoma and secreting germ cell tumors chemotherapies are playing an increasingly important role in t he interdisciplinary management. It can be expected that future development s of chemotherapeutic protocols and the introduction of new cytostatic subs tances will further improve the therapeutic outcome. Conclusions: The therapeutic endeavors of all those caring for children are aiming to study modifications of the therapeutic components in the interdi sciplinary approach in order to optimize the therapeutic strategies. In fut ure the affected children and young adolescents should be accrued for the f orthcoming cooperative, prospective trial HIT 2000 and ongoing trial SIOP C NS GCT 96, respectively, in order to provide the body of data supporting th e selection of novel and optimized approaches for future treatment strategi es.