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目的中枢神经系统非典型畸胎样/横纹肌样瘤(AT/RT)是少见的儿童胚胎性肿瘤,成人偶有发生。本研究通过报道1例儿童及1例成人AT/RT,探讨其临床病理特征、诊断及鉴别诊断方法。方法对2例AT/RT行HE及免疫组织化学染色观察,结合文献复习对其形态学及免疫表型特征进行分析。结果儿童(3岁)AT/RT 1例,MRI示右额颞叶混杂密度影;成人(33岁)AT/RT 1例,MRI示鞍区不均匀强化影,临床疑为垂体肿瘤。2例镜下均主要由横纹肌样细胞组成,伴有多少不等的原始神经外胚层肿瘤样成分。免疫组化显示Vim弥漫强阳性,GFAP、S-100、Syn、NF、CK、EMA、SMA、Desmin、MyoD1均有不同程度阳性,PLAP、HMB45阴性。结论 AT/RT是一高度恶性的中枢神经系统肿瘤,成人与儿童AT/RT在形态学及免疫组化特征上无明显差别,但成人在发病年龄、部位和预后上均与儿童有所不同,更易造成误诊,鉴别诊断侧重点也与儿童不同,需引起更多注意。
Atypical CNS atypical teratoid / rhabdomyosarcoma (AT / RT) is a rare childhood embryonal tumor that occurs occasionally in adults. This study reported 1 case of children and 1 adult AT / RT, to explore its clinicopathological features, diagnosis and differential diagnosis. Methods Two cases of AT / RT were stained with HE and immunohistochemistry, and their morphological and immunophenotypic characteristics were analyzed with literature review. Results A total of 1 AT / RT in children (3 years old) showed mixed right frontal lobe density and 1 case of AT / RT in adults (33 years old). MRI showed uneven enhancement of sellar region and clinical suspicion of pituitary tumor. 2 cases were mainly composed of rhabdomyosarcoma cells, with varying degrees of primitive neuroectodermal tumor-like components. Immunohistochemical staining showed that Vim was strongly and strongly diffusely positive. GFAP, S-100, Syn, NF, CK, EMA, SMA, Desmin and MyoD1 were all positive in different degrees, but negative in PLAP and HMB45. Conclusion AT / RT is a highly malignant central nervous system tumor. The morphological and immunohistochemical characteristics of AT / RT in adults and children have no significant difference. However, adults have different age, location and prognosis with children. More likely to cause misdiagnosis, differential diagnosis focus is also different with children, need to attract more attention.