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中枢神经系统非典型畸胎瘤样/横纹肌样瘤好发于婴幼儿后颅窝,其细胞形态学特征独特,肿瘤组织含有横纹肌样细胞、原始神经外胚层、上皮及间叶多向分化成分,染色体22q11.2上INI1肿瘤抑制基因缺失或突变是其分子生物学特征,结合影像学表现可准确诊断。AT/RT恶性度高,预后极差。目前,多采取以手术切除为主的综合治疗方法,未来研究的重点在于确定INI1功能并利用其进行有效干预性治疗。
Atypical teratoma / rhabdomyosarcoma in the central nervous system occurs in the posterior fossa of infants and young children. The cell morphology of the atypical teratoma / rhabdomyosarcoma is unique. The tumor contains rhabdomyosarcoma cells, primitive neuroectoderm, epithelial and mesenchyme differentiated components, The deletion or mutation of the INI1 tumor suppressor gene on chromosome 22q11.2 is a molecular biological feature that can be accurately diagnosed in conjunction with imaging findings. AT / RT is highly malignant and has a poor prognosis. At present, the surgical treatment to take more comprehensive treatment, the future focus of the study to determine the function of INI1 and use it for effective interventional treatment.