颅内原发性恶性外周神经鞘膜瘤4例临床病理分析  

Intracranial malignant peripheral nerve sheath tumors: report of 4 cases and review of literature

作　　者：吴卫平[1] 吴晋蓉[2] 何燕[2] 余波[2] 夏春[2] 印洪林[2] 石群立[2] 

机构地区：[1]江苏省启东市第二人民医院病理科 [2]南京军区南京总医院病理科,南京210002

出　　处：《诊断病理学杂志》2015年第8期456-458,463,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨颅内原发性恶性外周神经鞘膜肿瘤（MPNST）的临床及病理学特征,加深对这一疾病的认识。方法通过光镜及免疫组化对4例颅内恶性外周神经鞘膜肿瘤进行观察,同时复习相关文献。结果 4例MPNST中女性2例,男性2例,年龄4-61岁。临床表现包括面瘫、嗜睡、乏力伴呕吐,头晕、头痛伴单眼闭合不全,头痛伴行走不稳。巨检示肿瘤最大径4-12 cm,平均6.3 cm,边界不清;切面灰红色、鱼肉样,部分区域黏液变性,常伴出血、坏死。组织学示肿瘤主要由梭形细胞组成,呈弥漫、束状分布,疏密不一,可见黏液样背景。肿瘤细胞梭形、多形性,有明显的异型性。免疫组化示肿瘤细胞S-100（＋）,Ki-67呈高表达。3例随访6-24个月,患者死亡,1例肿瘤复发。结论颅内MPNST少见,预后差。确诊主要依病理学检查及免疫标记。手术切净肿瘤并辅以放疗及合理的化疗,可以延长患者的生存期。Objective To investigate the clinicopathologic feature of rare intracranial malignant peripheral nerve sheath tumor（ MPNST）. Methods Four cases of MPNST in the intracranial cavity were studied by microscopic observation and immunohistochemical staining. Results Of the 4 cases,2 were female,and others were male; the age of patients ranged from 4 to 61 years. The clinical manifestations included facial paralysis,sleepiness fatigue with vomiting,dizzinesss and headache with incomplete monocular closure,and headache with walking instability. Grossly,the largest diameter of tumors varied from 4. 0 to 12. 0 cm（ mean 6. 3 cm）. Tumor boundary wss not clear,the cut surface of the well-defined tumors was white and tan,part of the regional mucinous change,often with hemorrhage and necrosis. Pathologically,the tumor was composed of spindle cells with different density. The tumor cells were highly atypical. Some areas had myxoid background. Immunohistochemistry revealed that the tumor cells expressed S-100 protein and high proliferation index of Ki-67. All patients were followed up after operation for an average time of 24 months,3 cases died and 1 case recurred after 1year. Conclusion Intracranial MPNST is a rare tumor, with poor prognosis. The postoperative pathology and immunohistochemistry are the main basis for the diagnosis. Totally tumor resection is optional treatment followed by radiotherapy and chemotherapy. The survival time of patients may be prolong.

关 键 词：颅内 恶性外周神经鞘膜瘤 诊断 预后 

分 类 号：R739.43[医药卫生—肿瘤]