[Primary XXXX : Progress in the diagnostics and therapy].

@entity1499 @entity149 @entity2359 @entity706
BACKGROUND: Some important knowledge has recently been gained on @entity149 ( @entity149 ) despite its rarity. GOAL: This article summarizes the most relevant progress in the diagnostics and therapy of @entity149 and discusses future directions. MATERIAL AND METHODS: Reference articles in the English language literature were studied with respect to future approaches in @entity149 . RESULTS: New diagnostic methods in cerebrospinal fluid have been developed to facilitate @entity706 diagnosis; however, their value still has to be validated. A better immunohistological and molecular characterization of @entity149 will probably result in identification of new therapeutic targets. The only phase III trial for @entity149 completed so far did not demonstrate a survival advantage with whole brain irradiation after high-dose @entity1499 (HDMTX)-based chemotherapy as compared to chemotherapy alone. The optimal primary chemotherapy has not yet been established due to a lack of results from randomized trials. Non-comparative studies suggest a superiority of combined polychemotherapy over HDMTX monotherapy. Future therapeutic developments are directed towards consolidation of HDMTX-based induction chemotherapy with noncross-resistant conventional chemotherapy or high-dose chemotherapy with autologous stem cell transplantation. An important goal of all therapies for @entity149 is to avoid delayed @entity2359 . DISCUSSION: Further improvement of diagnostics and well-designed comparative studies, including new drugs when possible are still needed to define the optimal management of this still frequently prognostically unfavorable disease.
