Zhou Zixuan, Zhu Bingxin, Meng Qingming, Zhang Tong, Wu Yihao, Yu Rutong, Gao Shangfeng
Department of Neurosurgery, Institute of Nervous System Diseases, The Affiliated Hospital of Xuzhou Medical University, Xuzhou 221002, Jiangsu, China.
Department of Neurosurgery, Xuzhou Children's Hospital, Xuzhou Medical University, Xuzhou 221002, Jiangsu, China.
Explor Target Antitumor Ther. 2023;4(1):139-156. doi: 10.37349/etat.2023.00126. Epub 2023 Feb 28.
Medulloblastoma (MB) is the commonest primary malignant brain cancer. The current treatment of MB is usually surgical resection combined with radiotherapy or chemotherapy. Although great progress has been made in the clinical management of MB, tumor metastasis and recurrence are still the main cause of death. Therefore, definitive and timely diagnosis is of great importance for improving therapeutic effects on MB. In 2016, the World Health Organization (WHO) divided MB into four subtypes: wingless-type mouse mammary tumor virus integration site (WNT), sonic hedgehog (SHH), non-WNT/non-SHH group 3, and group 4. Each subtype of MB has a unique profile in copy number variation, DNA alteration, gene transcription, or post-transcriptional/translational modification, all of which are associated with different biological manifestations, clinical features, and prognosis. This article reviewed the research progress of different molecular pathology markers in MB and summarized some targeted drugs against these molecular markers, hoping to stimulate the clinical application of these molecular markers in the classification, diagnosis, and treatment of MB.
髓母细胞瘤(MB)是最常见的原发性恶性脑癌。目前MB的治疗通常是手术切除联合放疗或化疗。尽管MB的临床管理已取得巨大进展,但肿瘤转移和复发仍是主要死因。因此,明确且及时的诊断对于提高MB的治疗效果至关重要。2016年,世界卫生组织(WHO)将MB分为四种亚型:无翅型小鼠乳腺肿瘤病毒整合位点(WNT)、音猬因子(SHH)、非WNT/非SHH 3组和4组。MB的每个亚型在拷贝数变异、DNA改变、基因转录或转录后/翻译后修饰方面都有独特的特征,所有这些都与不同的生物学表现、临床特征和预后相关。本文综述了MB中不同分子病理标志物的研究进展,并总结了一些针对这些分子标志物的靶向药物,希望能推动这些分子标志物在MB分类、诊断和治疗中的临床应用。