中枢神经系统胚胎性肿瘤是高度侵袭性恶性肿瘤，主要影响婴幼儿，即使给予积极的治疗策略，预后仍然很差。2016版世界卫生组织(WHO)分类将疾病遗传信息与组织病理学相整合，使这些肿瘤的分类和诊断更加精确。在新版分类中，中枢胚胎性肿瘤包括最常见的髓母细胞瘤，以及罕少见的多层菊形团胚胎性肿瘤，n C19MC变异型、多层菊形团胚胎性肿瘤，非特指、髓上皮瘤、中枢神经系统神经母细胞瘤、中枢神经系统神经节细胞神经母细胞瘤、中枢胚胎性肿瘤，非特指、非典型性畸胎瘤/横纹肌样肿瘤、中枢胚胎性肿瘤伴横纹肌样特点。对这些罕见肿瘤生物学本质的进一步研究及探索，将为新的治疗方法提供科学依据。n “,”Embryonal tumors of central nervous system (CNS) are highly invasive malignant tumors that mainly affect infants.Even if the current active treatment strategies are given, the prognosis is still very poor.The 2016 World Health Organization(WHO) classification integrates the genetic information of diseases with histopathology, so as to make the classification and diagnosis of these tumors more accurate.In the new classification, embryonal tumors of CNS include the most common medulloblastoma, and the rare embryonal tumor with multilayered rosettes, n C19MC-altered, embryonal tumor with multilayered rosettes, not otherwise specified (NOS), medulloepithelioma, CNS neuroblastoma, CNS ganglioneuroblastoma, CNS embryonal tumor, NOS, atypical teratoid rhabdoid tumor, and CNS embryonal tumor with rhabdoid features.Further study and exploration on the biological nature of these rare brain tumors will provide scientific basis for new treatment methods.n