正中神经恶性神经束膜瘤临床病理观察

Malignant perineurioma of median nerve:a clinicopathologic observation

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 perineurioma. Methods Microscopy and immunohistochemistry were applied in one case of malignant perineurioma of median nerve, analyzing the clinicopathologic features, and the literatures were also reviewed. Results The neoplasm origined from the perineuronal cell, appearing epithelioid, partly whorled arrangement. At the rim of the neoplasm, rudimental nerve axes were found. Mitotic figures and atypia were found in the neoplastic cells. The immunostaining showed that the neoplastic cells were positive for CK, EMA, vimentin, and also CD34, but negative for S-100. Conclusion Malignant perineuroma is a rare neoplasm with poor prognosis, which should be differentiated from MPNST, plexiform nerve fibroma, epithelioid sarcoma and synovial sarcoma, and immunohistochemistry helps in the differential diagnosis, combined with morphological features.