脑膜恶性孤立性纤维性肿瘤临床病理学观察

目的:探讨脑膜恶性孤立性纤维性肿瘤的临床病理特征、核磁共振特点、鉴别诊断及治疗和预后。方法:报道一例脑膜恶性孤立性纤维性肿瘤临床病理、影像学资料并复习文献。结果:患者,男性,65岁,临床主要表现为四肢乏困,无力,记忆力差,MRI示左颞枕叶可见约8.5cm×6.5cm×6cm的囊实性病灶,术中发现肿瘤累及脑膜,与周围脑组织界限清楚,送检肿瘤组织灰白色均质状,有囊性变。镜检:肿瘤由交替性分布的细胞丰富区和细胞稀疏区组成,细胞排列呈条束状、席纹状或无结构样,部分区域呈血管外皮瘤样结构,部分区域细胞丰富密集,有异型性,核分裂象多见(>4个/10HPF),未见明显坏死。免疫表型:肿瘤细胞Vimentin、CD99、bcl-2、CD34弥漫阳性,p53弱阳性,Ki-67>10%。病理诊断:左颞枕叶脑膜恶性孤立性纤维性肿瘤。结论:脑膜恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并与脑的其它梭形细胞肿瘤鉴别。治疗以手术完整切除为主,可预防性放疗并密切随访。

Clinicopathological observation of malignant solitary fibrous tumor of the meninges

Objective: To investigate the clinicopathologicl MRI characteristics,differential diagnosis,treatment and prognostic of malignant solitary fibrous tumor of the meninges.Methods: One case of malignant SFT of the meninges was reported,the clinical features,immunohistochemical analysis and imaging appearance were observed.Results: The patient was a 65-year-old male presenting with four limbs were weakness,the memory was bad,MRI showed the left temporoccipital lobe approximately 8.5cm×6.5cm×6cm cystic solid mass.In operation was the tumor attached to the meninges of discovered obviously,the tumor was well circumscribed with the periphery brain organization.General sample showed grayish white isotropic shape,and cystic changes.Histopathologically: The tumor was composed of the spindle-shaped cells with fascicular,stroform or patternless pattern,disposed in sfascicular arrangement and separated by prominent eosinophilic bands of collagen,and showed a haemangiopericytoma-like structure.the nuclear mitoses(>4 /10HPF),no obvious necrosis.Immunochemistry results showed the tumor cells were diffusely positive for vimentin,CD34,CD99,bcl-2.ki-67 labelling index was more than 10%.The pathological diagnosis was malignant SFT of meninges.Conclusion:The malignant SFT of meninges is very rare.Diagnosis is based on its pathological morphology and immunohistochemistry.Differential diagnosis includes other spindle-shaped celltumors of the meninges.The treatment is complete surgical resection,and a long-term follow-up is required.