S. Schmandt et J. Kuhl, CHEMOTHERAPY AS PROPHYLAXIS AND TREATMENT OF MENINGOSIS IN CHILDREN LESS-THAN 3 YEARS OF AGE WITH MEDULLOBLASTOMA, Journal of neuro-oncology, 38(2-3), 1998, pp. 187-192
The outcome of children less than 3 years of age with medulloblastoma
has been poor in comparison to older children. The cure rates were bel
ow 30%, and the quality of life for cured children was frequently redu
ced by a complex syndrome of long-term sequelae including intellectual
retardation and growth hormone deficiency. Due to the deleterious sid
e-effects of radiotherapy in very young children chemotherapy has play
ed an important role in this group of patients. Firstly, chemotherapy
should improve their survival rate. Secondly, it should allow dose sed
uction of craniospinal irradiation and a smaller involved field. With
the goal of improving quality of life radiotherapy should be delayed o
r even replaced by postoperative chemotherapy. The EFS of low-risk pat
ients steadily improved and is now as high as at least 50%. Since most
patients of this group do not need radiation, treatment-related long-
term sequelae are minimal. High-risk patients, by contrast, with metas
tatic disease or measurable postoperative tumor still have a very disa
ppointing progression-free survival in a range below 30% at 3 to 3 yea
rs in all large studies. Therefore prevention and effective therapy of
meningosis, as well as a good response to induction chemotherapy, are
essential for the outcome. Strategies to increase the efficacy of con
ventional treatment modalities in high-risk patients are under investi
gation. Recently, interesting results have been published on high-dose
chemotherapy followed by autologous stem cell rescue and intraventric
ular administration of the alkylating agent mafosfamide.