Molecular Classification of Medulloblastoma.
Molecular Classification of Medulloblastoma.
复制标题
髓母细胞瘤的分子分类。
DOI：
10.2176/nmc.ra.2016-0016
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发表时间：
2016-11-15
影响因子：
1.9
通讯作者：
Kanemura Y
中科院分区：
文献类型：
--
作者：
Kijima N;Kanemura Y
髓母细胞瘤是儿童最常见的恶性脑肿瘤之一。目前的标准治疗方案包括手术切除、颅脊髓照射和辅助化疗。虽然这些治疗有可能增加70-80% MB患者的生存率，但它们也与严重的治疗诱导的发病率相关。目前MB的风险分层是基于临床因素，包括就诊时的年龄、转移状态和切除后残留肿瘤的存在。此外，最近的基因组研究表明，MB由至少四个不同的分子亚组组成：WNT，音刺猬（SHH），第3组和第4组。WNT和SHH MB的特征分别在于WNT和SHH信号通路中的畸变。与其他MB相比，WNT MB具有最佳预后，而SHH MB具有中等预后。与第3组和第4组MB相关的潜在信号通路尚未确定。第3组MB通常与转移有关，导致预后不良，而第4组有时与转移有关，预后中等。第4组是最常见的MB，占所有MB的35%。这些发现表明MB是一种异质性疾病，MB亚组具有不同的分子、人口统计学和临床特征。MB的分子分类正在重新定义MB患者的风险分层，并有可能确定MB治疗的新治疗策略。
Medulloblastoma (MB) is one of the most frequent malignant brain tumors in children. The current standard treatment regimen consists of surgical resection, craniospinal irradiation, and adjuvant chemotherapy. Although these treatments have the potential to increase the survival of 70–80% of patients with MB, they are also associated with serious treatment-induced morbidity. The current risk stratification of MB is based on clinical factors, including age at presentation, metastatic status, and the presence of residual tumor following resection. In addition, recent genomic studies indicate that MB consists of at least four distinct molecular subgroups: WNT, sonic hedgehog (SHH), Group 3, and Group 4. WNT and SHH MBs are characterized by aberrations in the WNT and SHH signaling pathways, respectively. WNT MB has the best prognosis compared to the other MBs, while SHH MB has an intermediate prognosis. The underlying signaling pathways associated with Group 3 and 4 MBs have not been identified. Group 3 MB is frequently associated with metastasis, resulting in a poor prognosis, while Group 4 is sometimes associated with metastasis and has an intermediate prognosis. Group 4 is the most frequent MB and represents 35% of all MBs. These findings suggest that MB is a heterogeneous disease, and that MB subgroups have distinct molecular, demographic, and clinical characteristics. The molecular classification of MBs is redefining the risk stratification of patients with MB, and has the potential to identify new therapeutic strategies for the treatment of MB.