恶性外周神经鞘膜瘤10例临床病理学分析及文献复习-东南大学学报医学版

恶性外周神经鞘膜瘤10例临床病理学分析及文献复习

单位：1. 川北医学院病理教研室, 四川南充 637000;
2. 川北医学院附属医院消化内科, 四川南充 637000

分类号：R739.43

出版年·卷·期（页码）：2015·34·第三期（386-389）

摘要：

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 malignant 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 features 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 epidural 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. Histologically, It was appeared as fusocellular sarcoma, and 1 case was accompanied with necrosis, 2 cases with heterologous 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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