Jozwiak Jaroslaw, Grajkowska Wieslawa, Wlodarski Pawel
Department of Histology and Embryology, Center for Biostructure Research, Medical University of Warsaw, Warsaw, Poland.
Med Res Rev. 2007 Nov;27(6):869-90. doi: 10.1002/med.20088.
Medulloblastoma is the most common malignant tumor of the cerebellum in children, with a tendency to metastasize via CSF pathway. Survival rate varies depending on several factors, but is rather favorable, with radiotherapy as the treatment of choice. Irradiation of the craniospinal axis results, however, in severe neuropsychological and psychosocial impairments pertaining to memory, attention, motor functioning, language, and visuospatial abilities. Precise mechanisms underlying the formation of medulloblastoma are still unclear, but implication of at least three signaling molecules is postulated: insulin-like growth factor-I, WNT, and Sonic hedgehog. Thanks to increasing knowledge on the cellular mechanisms contributing to tumor formation, it is possible to propose new therapies that could replace radiotherapy or allow decreasing irradiation doses. The current review presents recent developments in medulloblastoma pathophysiology research and proposed inhibitors that could constitute good candidates for further pharmacological research.
髓母细胞瘤是儿童小脑最常见的恶性肿瘤,有通过脑脊液途径转移的倾向。生存率因多种因素而异,但相当乐观,放射治疗是首选治疗方法。然而,全脑全脊髓照射会导致严重的神经心理和社会心理障碍,涉及记忆、注意力、运动功能、语言和视觉空间能力。髓母细胞瘤形成的精确机制仍不清楚,但推测至少有三种信号分子参与其中:胰岛素样生长因子-I、WNT和音猬因子。由于对肿瘤形成的细胞机制的了解不断增加,有可能提出新的疗法来替代放射治疗或减少照射剂量。本综述介绍了髓母细胞瘤病理生理学研究的最新进展以及提出的抑制剂,这些抑制剂可能是进一步药理学研究的良好候选者。