Byrd Tiara, Grossman Robert G, Ahmed Nabil
Department of Pediatrics, Baylor College of Medicine, Center for Cell and Gene Therapy, Houston, Texas 77030, USA.
Pediatr Hematol Oncol. 2012 Sep;29(6):495-506. doi: 10.3109/08880018.2012.698372. Epub 2012 Jun 28.
Medulloblastoma (MB) is a cancer of the cerebellum and the most common primary pediatric malignancy of the central nervous system. Classified as a primitive neural ectoderm tumor; it is thought to arise from granule cell precursors in the cerebellum. The standard of care consists of surgery, chemotherapy and age-dependent radiation therapy. Despite aggressive multimodality therapy; approximately 30% of MB patients remain incurable. Moreover, for long-term survivors, the treatment related sequelae are often debilitating. Side effects include cerebellar mutism, sterility, neurocognitive deficits, and a substantial risk of developing secondary cancers. In a quest for more effective and targeted therapies, scientists have begun to investigate the biological events that not only initiate but also sustain the malignant phenotype in MB. Of particular interest is, the role of the tumor microenvironment in tumor pathogenesis. This review seeks to highlight several key processes observed in cancer biology, particularly the involvement of the tumor microenvironment, with relevant examples from MB.
髓母细胞瘤(MB)是一种小脑癌症,也是中枢神经系统最常见的原发性儿科恶性肿瘤。它被归类为原始神经外胚层肿瘤,被认为起源于小脑的颗粒细胞前体。治疗标准包括手术、化疗和根据年龄进行的放射治疗。尽管采用了积极的多模态治疗,但仍有大约30%的MB患者无法治愈。此外,对于长期存活者来说,与治疗相关的后遗症往往使人虚弱。副作用包括小脑缄默症、不育、神经认知缺陷以及发生继发性癌症的重大风险。为了寻求更有效和有针对性的治疗方法,科学家们已经开始研究不仅启动而且维持MB恶性表型的生物学事件。特别令人感兴趣的是肿瘤微环境在肿瘤发病机制中的作用。本综述旨在强调癌症生物学中观察到的几个关键过程,特别是肿瘤微环境的参与,并列举MB的相关实例。