Chatterjee Abhishek, Maitre Madan, Dasgupta Archya, Sridhar Epari, Gupta Tejpal
Neuro-oncology Disease Management Group, Homi Bhabha National Institute (HBNI), Tata Memorial Centre, Mumbai, India.
Methods Mol Biol. 2022;2423:215-235. doi: 10.1007/978-1-0716-1952-0_19.
Medulloblastoma is a highly aggressive "small round blue cell tumor" of the posterior fossa predominantly seen in children. Historically aggressive multimodality regimens have achieved encouraging outcomes with the caveat of severe long-term toxicities. The last decade has unleashed a revolution in terms of evolved understanding of this heterogeneous disease entity in terms of molecular biology. Medulloblastoma as of today is grouped into one of four canonical molecular subgroups (WNT, SHH, Group 3, and Group 4) each characterized by different putative cells of origin, characteristic aberrations at the molecular level, radiogenomics, and outcomes. Our understanding continues to grow in this regard. The future promises much in terms of personalized medicine in tailoring therapy to the needs of individual patients based on their clinical and molecular profile in order to maximize individual and population based outcomes at the cost of minimizing toxicity.
髓母细胞瘤是一种高度侵袭性的“小圆蓝细胞瘤”，主要发生于儿童后颅窝。从历史上看，积极的多模式治疗方案取得了令人鼓舞的结果，但存在严重长期毒性的问题。在过去十年中，我们对这种异质性疾病实体的分子生物学认识有了很大进展。如今，髓母细胞瘤被分为四个典型分子亚组之一（WNT、SHH、3组和4组），每个亚组都有不同的假定起源细胞、分子水平的特征性畸变、放射基因组学和预后。我们在这方面的认识仍在不断发展。未来，基于患者的临床和分子特征为个体患者量身定制治疗方案的精准医学有望实现，以在将毒性降至最低的前提下，最大化个体和群体的治疗效果。
