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目的探讨恶性神经束膜瘤的病理形态特点、诊断及鉴别诊断。方法应用光学显微镜及免疫组化方法观察1例正中神经恶性神经束膜瘤(MP),并进行临床病理分析及复习文献。结果肿瘤来自神经束膜细胞,呈上皮样排列,局部可见旋涡状排列方式,在肿瘤周边可见残留的神经轴索;肿瘤细胞异型性明显,核分裂象多见。免疫组化显示肿瘤细胞表达上皮标记物,CK和EMA均强(+),CD34(+),而S-100(-)。结论恶性神经束膜瘤罕见,预后较差,临床需注意与恶性神经鞘瘤、丛状神经纤维瘤、上皮样肉瘤、滑膜肉瘤鉴别,可以根据其形态学特点及与神经的关系结合免疫组化鉴别。
Objective To investigate the pathological features, diagnosis and differential diagnosis of malignant neuroblastoma. Methods One case of malignant neurocytoma (MP) was observed by light microscopy and immunohistochemistry, and the clinical and pathological analysis and review of the literature were performed. Results The tumors originated from perineal epithelial cells and arranged in epithelial order with some vortex-like arrangement. The residual axons were visible around the tumor. The atypia of the tumor cells was obvious and the mitotic figures were more common. Immunohistochemistry showed that tumor cells expressed epithelial markers with strong CK and EMA (+), CD34 (+), and S-100 (-). Conclusions Malignant medulloblastoma is rare and has a poor prognosis. It should be distinguished from malignant schwannoma, plexiform neurofibroma, epithelioid sarcoma and synovial sarcoma according to its morphological characteristics and the relationship with the nerve Discrimination.