多能干细胞为研究疾病中人类特异性方面提供了独特的途径，是肿瘤研究中高度多功能的工具。肿瘤发生过程和发育程序常常共享重叠的转录组和表观遗传学特征，这些特征可以重新激活诱导多能干细胞中。随着脑器官的出现，再现大脑发育和结构的能力已经极大地提高，使体外模型更加现实，因此更适合于生物医学建模。本综述重点介绍了人类多能干细胞模拟脑和神经嵴肿瘤的最新研究和当前挑战，并呼吁进行更加严格的质量控制和开发罕见肿瘤亚型的模型。© 作者（们）2023。由牛津大学出版社代表神经肿瘤学会出版。

Pluripotent stem cells offer unique avenues to study human-specific aspects of disease and are a highly versatile tool in cancer research. Oncogenic processes and developmental programs often share overlapping transcriptomic and epigenetic signatures, which can be reactivated in induced pluripotent stem cells. With the emergence of brain organoids, the ability to recapitulate brain development and structure has vastly improved, making in vitro models more realistic and hence more suitable for biomedical modeling. This review highlights recent research and current challenges in human pluripotent stem cell modeling of brain and neural crest neoplasms, and concludes with a call for more rigorous quality control and for the development of models for rare tumor subtypes.© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.