Rawal Zeal D, Upadhyay Vinal A, Patel Dipak D, Trivedi Trupti I
Clinical Carcinogenesis Laboratory, Department of Cancer Biology, The Gujarat Cancer & Research Institute, Civil Hospital Campus, Ahmedabad, Gujarat, India.
Department of Neuro Oncology, The Gujarat Cancer & Research Institute, Civil Hospital Campus, Ahmedabad, Gujarat, India.
J Pediatr Neurosci. 2020 Jul-Sep;15(3):175-182. doi: 10.4103/jpn.JPN_166_18. Epub 2020 Nov 6.
Medulloblastoma (MB) is a devastating illness with unmet therapeutic needs, predominantly cytotoxic and nontargeted approaches. Survivors of MB also suffer from severe treatment-related effects of radiation and cytotoxic chemotherapy keeping mortality rate significant. Recently, four distinct molecular subgroups of MB have been identified (WNT [wingless], SHH [sonic hedgehog], Group 3, and Group 4). Novel subgroup-specific therapies are being explored in the daily treatment of patients as a clinical trial and are an important challenge in the near term for the pediatric neurooncology society. Epigenetic modifiers are also recurrently affected in MB suggesting that epigenetic therapy can be considered in a subset of patients. Moreover, a hint on forefront procedure; tracer of cancer's genetic information entitled "liquid biopsy" in MB is described. This review examines the recent scientific progress in MB research, with a focus on the genes, pathways that drive tumorigenesis and the advances in conventional and targeted therapy. The identification of subgroup-specific, actionable therapeutic targets has the potential to revolutionize therapy for patients with MB and results in significantly enriched overall survival.
髓母细胞瘤(MB)是一种治疗需求未得到满足的毁灭性疾病,主要采用细胞毒性和非靶向治疗方法。MB幸存者还遭受放疗和细胞毒性化疗带来的严重治疗相关影响,死亡率仍然很高。最近,已确定了MB的四个不同分子亚组(WNT[无翅型]、SHH[音猬因子]、3组和4组)。作为临床试验,新型亚组特异性疗法正在患者的日常治疗中进行探索,并且是儿科神经肿瘤学会近期面临的一项重要挑战。表观遗传修饰因子在MB中也经常受到影响,这表明可以在一部分患者中考虑表观遗传治疗。此外,还描述了前沿程序的一个提示;在MB中名为“液体活检”的癌症遗传信息追踪器。本综述探讨了MB研究的最新科学进展,重点关注驱动肿瘤发生的基因、途径以及传统和靶向治疗的进展。亚组特异性、可操作治疗靶点的确定有可能彻底改变MB患者的治疗方式,并显著提高总生存率。