目的：探讨恶性外周神经鞘膜瘤（ MPNST ）的组织起源、病理学形态特点及鉴别诊断。方法：应用光学显微镜及免疫组化方法观察10例MPNST的病理形态特点及免疫组织化学表型，并进行临床病理学分析及文献复习。结果：10例MPNST中男7例，女3例；年龄31～71岁，平均年龄45．6岁；肿瘤发生部位为四肢（3例）、消化道（2例）、腹腔（2例）、鼻腔（1例）、腹股沟（1例）、硬膜外（1例）；肿瘤直径为1．5～15 cm，瘤体呈结节状，部分有包膜，其中1例肿瘤伴有出血、坏死。肿瘤组织学表现为梭形细胞肉瘤，1例伴有坏死，2例伴有异源性成分。免疫组化检测显示肿瘤细胞表达神经标志物，S-100和NSE阳性，2例伴有异源性成分者Myoglobin阳性。结论：MPNST组织结构变异大，可以通过肿瘤的发生部位、形态学特点，并结合免疫组化染色与纤维肉瘤、平滑肌肉瘤、滑膜肉瘤、上皮样肉瘤等相鉴别。%Objective:To investigate the origin of tissue , pathological features and differential diagnosis of malig-nant peripheral nerve sheath tumor ( MPNST).Methods: The pathological morphology and immunohistochemical features were observed in 10 cases of MPNST by microscopy and immunohistochemistry .The clinicopathologic fea-tures was analyzed and the literatures were reviewed .Results:Among 10 cases of MPNST , there were 7 male and 3 female with an age range from 31 to 71 years old, average 45.6 years.Three cases were discovered in limbs , 2 cases in alimentary canal , 2 cases in abdominal cavity , 1 case in nasal cavity , 1 case in groin, and 1 case in epi-dural respectively.The diameter of tumors was ranged from 1.5-15 cm.The tumors were appeared as nodules , and part of tumors had capsule , additionally , 1 case of tumor was accompanied with hemorrhage and necrosis .Histolog-ically, It was appeared as fusocellular sarcoma , and 1 case was accompanied with necrosis , 2 cases with heterolo-gous components .The immunostaining showed that the neoplastic cells were positive for S-100 and NSE, cells in 2 cases with heterologous compents were positive for Myoglobin .Conclusion:The variability of organization structure are found in neoplastic cells , and the MPNST should be differentiated from fibrosarcoma , leiomyosarcoma ,synovial sarcoma and epithelioid sarcoma by immunohistochemistry , combined with the morphological features of tumor and the location of the tumor occurrence .
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