成人髓母细胞瘤仅仅是儿童髓母细胞癌的对应物吗?

Glioma Pub Date : 2020-07-01 DOI:10.4103/glioma.glioma_24_20
G. Wong, K. Li, M. Poon, H. Ng
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引用次数: 0

摘要

髓母细胞瘤是儿童最常见的恶性脑肿瘤之一。在过去的十年中,随着四种不同的分子群的确定,对其的理解和治疗取得了迅速的进展。相比之下,成人髓母细胞瘤是一种罕见的实体,占成人中枢神经系统肿瘤的不到1%,在其遗传景观和临床管理方面都未得到充分研究。成人成神经管细胞瘤与儿科成神经管细胞瘤有许多不同之处,这与临床医生和生物学家有关。与儿童不同,成人髓母细胞瘤通常位于小脑外侧,很少转移,通常在5年以上复发。成人成神经管细胞瘤分子群的分布和生存结果与儿童成神经管细胞瘤不同,其中声刺猬激活组是成人中主要的和研究最多的组。成人髓母细胞瘤也表现出该年龄组特有的细胞遗传学和突变特征,例如端粒酶逆转录酶启动子突变的高频率以及MYC和MYCN扩增的缺乏。成人成神经管母细胞瘤的临床试验需要考虑成人和儿童成神经管母细胞瘤的临床和生物学差异,例如,通过在成人SHH成神经管母细胞瘤中使用平滑抑制剂来降低直接采用儿科化疗方案所产生的毒性。本文综述了成人成神经管细胞瘤的临床特点、分子类群、遗传特征和治疗方法,并重点介绍了成人成神经管细胞瘤与儿童成神经管细胞瘤的区别。
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Is adult medulloblastoma merely the counterpart of pediatric medulloblastoma?
Medulloblastoma is one of the most common pediatric malignant brain tumors. Its understanding and treatment have advanced rapidly over the decade, with the identification of four distinct molecular groups. In contrast, adult medulloblastoma is a rare entity that accounts for <1% of adult central nervous system tumors, and is understudied in both its genetic landscape and clinical management. Adult medulloblastomas demonstrate many differences from pediatric medulloblastomas that are relevant to clinicians and biologists. Unlike its pediatric counterpart, adult medulloblastomas are typically located laterally in the cerebellum, are seldom metastatic, and commonly relapse beyond 5 years. The distribution and survival outcomes of molecular groups in adult medulloblastoma differ from those in pediatric medulloblastoma, with the sonic hedgehog-activated group being the predominant and most well-studied group in adults. Adult medulloblastomas also exhibit cytogenetic and mutational characteristics unique to this age group, such as the high frequency of telomerase reverse transcriptase promoter mutations and the paucity of MYC and MYCN amplifications. Clinical trials for adult medulloblastoma need to take into account the clinical and biological differences between adult and pediatric medulloblastomas, for example through the use of smoothened inhibitors in adult SHH medulloblastomas to lower the toxicities resulting from direct adoption of pediatric chemotherapeutic regimens. This review summarizes the clinical characteristics, molecular groups, genetic features, and treatment of adult medulloblastoma, with a focus on its differences from pediatric medulloblastoma.
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