Department of Neurological Surgery, University of California, Los Angeles, 695 Charles E. Young Drive South, Gonda 3357, Los Angeles, California 90095-1761, USA.
J Clin Neurosci. 2011 Jul;18(7):886-90. doi: 10.1016/j.jocn.2011.01.001.
Medulloblastomas (MB) are highly aggressive primitive neuroectodermal tumors (PNET) usually located in the posterior fossa. Current treatment for MBs, which includes a combination of surgery, chemotherapy and radiation, remain challenging especially in younger patients. However, advances in the understanding of regulatory pathways in cerebellar development have elucidated possible areas of dysfunction involved in tumorigenesis. Multiple studies have demonstrated the importance of the sonic hedgehog, Wnt, and Notch pathways in MB pathogenesis at the molecular level. While staging and prognosis are often based on the Chang classification system, future algorithms will involve identifying molecular markers in order to allow for more specific risk stratifications of various MB subtypes and provide improved correlation with staging and prognosis. Future development of novel therapies that target the heterogeneity of MB and are tailored to the tumor's unique molecular profile may yield improved outcomes for these patients.
髓母细胞瘤（MB）是高度侵袭性的原始神经外胚层肿瘤（PNET），通常位于后颅窝。目前的治疗方法包括手术、化疗和放疗的综合治疗，但对于年轻患者来说仍然具有挑战性。然而，对小脑发育调控途径的认识的进步已经阐明了肿瘤发生过程中可能涉及的功能障碍区域。多项研究表明， sonic hedgehog、Wnt 和 Notch 通路在 MB 的发病机制中具有重要的分子水平作用。虽然分期和预后通常基于 Chang 分类系统，但未来的算法将涉及识别分子标志物，以便能够对各种 MB 亚型进行更具体的风险分层，并与分期和预后更好地相关。针对 MB 的异质性并针对肿瘤独特的分子特征量身定制的新型治疗方法的未来发展，可能会为这些患者带来更好的结果。
