PDF(Pubmed)
Abstract：
Choroid plexus carcinomas (CPC) are early childhood cancers characterized by loss of TP53 function and poor survival. We are analyzing data on TP53 status, survival, and second cancers from the largest cohort of CPC receiving chemotherapy followed by consolidation with marrow-ablative chemotherapy (HDCx). Additionally, we discuss the rationale for targeted therapies for CPC patients. Currently, 8 of the 13 with Li-Fraumeni Syndrome-associated CPC were treated and continued CPC-free, indicating that HDCx improves CPC-free survival in young children with TP53-mutated CPC. These data justify the inclusion of HDCx in the planned prospective international trial for children with TP53-mutated CPC.
摘要：
脉络丛癌(CPC)是以TP53功能丧失和低生存率为特征的早期儿童癌症。我们正在分析TP53状态的数据，生存,其次是来自接受化疗的最大CPC队列的癌症，其次是合并骨髓消融化疗（HDCx）。此外,我们讨论了CPC患者靶向治疗的基本原理.目前,与Li-Fraumeni综合征相关的CPC的13人中有8人接受了治疗，并继续无CPC，表明HDCx改善TP53突变的CPC幼儿的无CPC生存率。这些数据证明将HDCx纳入TP53突变CPC儿童的计划前瞻性国际试验中。
