Medulloblastoma in childhood: new biological advances

被引:194
作者
Crawford, John R. [1 ,3 ]
MacDonald, Tobey J. [1 ,2 ,4 ]
Packer, Roger J. [1 ,3 ,4 ]
机构
[1] George Washington Univ, Div Neurol, Washington, DC 20010 USA
[2] George Washington Univ, Div Oncol, Washington, DC USA
[3] George Washington Univ, Childrens Natl Med Ctr, Dept Neurol, Washington, DC USA
[4] George Washington Univ, Childrens Natl Med Ctr, Dept Pediat, Washington, DC USA
关键词
D O I
10.1016/S1474-4422(07)70289-2
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Medulloblastoma is the most common embryonal tumour in children. Patients with medulloblastoma are currently staged as average-risk or poor-risk on the basis of clinical findings. With current multimodality therapy, nearly 90% of children with average-risk, non-disseminated medulloblastoma have 5-year event-free survival, and those with high-risk disease have a 60-65% survival rate; however, the outcome for younger children, particularly infants, is worse. Children who survive medulloblastoma are at risk of long-term sequelae related to the neurological effects of the tumour, surgery, or radiotherapy, and the additive effects of chemotherapy. Molecular biology has changed our understanding of medulloblastoma and has implications for diagnostic stratification and treatment. As newer biological agents are translated from the lab to the bedside, clinicians need to understand the fundamental signalling pathways that are targeted during therapy. Greater understanding of the molecular biology of medulloblastoma is needed so that more children can be cured or have an improved quality of life.
引用
收藏
页码:1073 / 1085
页数:13
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