J Clin Oncol:髓母细胞瘤复发后的临床预后和分子变异模式

2021-01-28 MedSci原创 MedSci原创

本研究旨在调研复发性髓母细胞瘤的临床预后,对比患者匹配的诊断性肿瘤和复发性肿瘤的分子特征。

髓母细胞瘤(medulloblastoma)是一种主要发生于14岁以下儿童后颅窝的高度恶性的胶质瘤。髓母细胞瘤复发是导致儿童癌症相关死亡的一个主要原因。本研究旨在调研复发性髓母细胞瘤的临床预后,对比患者匹配的诊断性肿瘤和复发性肿瘤的分子特征

本研究对SJMB03(NCT00085202)和SJYC07(NCT00602667)试验招募的经历了髓母细胞瘤复发的儿童和婴儿进行临床预后分析,包括复发的解剖和时间模式及复发后的存活率。

复发时间

SJMB03试验329位受试患儿中有72位(22%)、SJYC07试验79位患儿中有52位(66%)经历了复发,主要位第三组和无翼肿瘤模式。虽然大多数患者为远处病灶(79%),仍有38%的声波刺猬(SHH)髓母细胞瘤为孤立性局部复发。复发时间和复发后的存活率因分子亚组而异,其中第4组肿瘤患者的潜伏期更长。SJYC07试验中既往未放疗过的患者复发后进行放疗与长存活期相关

复发后的存活率

在127位有患者匹配的肿瘤对的患者中,9位(7%)继发了非髓母细胞瘤中枢神经系统恶性肿瘤。其他患者的亚组(96%)和亚型(80%)基本保持稳定。第3组和第4组肿瘤之间观察到了罕见的亚组差异,与MYC、MYCN和FBXW7相关遗传变异一致。

总之,复发性髓母细胞瘤的临床行为必须根据前期疗法和分子分类来进行情景化的研究。复发后放疗的疗效取决于患者年龄和既往是否采用过放疗。复发时分子保守性的程度和模式因分组而异。复发治疗组织可用于验证分子靶点并筛查隐匿性继发性恶性肿瘤。

原始出处:

Rahul Kumar, et al. Clinical Outcomes and Patient-Matched Molecular Composition of Relapsed Medulloblastoma. Journal of Clinical Oncology. January 27, 20221.

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    2021-01-28 anti-cancer

    谢谢梅斯分享这么多精彩信息

    0

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