中枢神经系统孤立性纤维瘤的显微外科手术治疗

Microsurgical treatment of solitary fibrous tumor in the central nervous system

目的探讨中枢神经系统孤立性纤维瘤（cSFT）的临床特征、影像学和病理学表现以及治疗策略，以提高对该肿瘤的诊断和治疗。方法对2010年6月至2016年7月收治的14例病例进行回顾性分析，对患者的临床特征、治疗及预后等方面予以总结和分析。结果14例经显微手术治疗的中枢神经系统孤立性纤维瘤患者中．首次报道了发生于枕骨大孔区1例．丰富了对本疾病的认识。肿瘤全切除者12例，未全切除者2例；病理学诊断良性肿瘤11例．低度恶性肿瘤3例：镜检瘤细胞致密区与疏松区交替性分布，细胞间夹杂胶原纤维。免疫组化显示所有病例Vimentin、CD34、Bel-2阳性，12例病例CD99阳性。随访6。49个月，全切除的12例无肿瘤复发．未全切除的2例均出现复发。结论中枢神经系统孤立性纤维瘤是一种罕见疾病，MRI表现有～定特征性．确诊需依靠病理组织学及免疫组织化学检查。肿瘤全切除预后良好，对于未全切除的肿瘤患者建议术后行放射治疗．化学治疗疗效尚不明确。

Objective To explore the clinicopathological features, imaging manifestation and treatment of solitary fibrous tumor in the central nervous system（cSFT）, in order to improve the diagnosis and treatment. Methods Fourteen cases of cSFT that had been received microsurgery treatment from June, 2010 to July, 2016 were analyzed retrospectively. The clinicopathologieal features, treatment and prognosis were concluded and analyzed. Results In 14 patients with cSFT treated with microsurgery, and 1 case in the foramen magnum region for the first time, and en- riched the understanding of this disease. Of 14 patients with cSFT, 12 received total resection and 2 subtotal or part. The eSFT were pathologically diagnosed as benign tumors in 11 patients and low grade eSFT in 3. The microscopical presence of spindle-shaped tumor cells arranged in fascicles that alternate hypocellular and hypercellular areas sepa- rated from each other by eollagenous fibre. Immunohistochemistry was positive for Vimentin, and CD34, Bcl-2 of 14 patients, and positive for CD99 in 12 patients. Followed-up time was from 6 to 49 months. Twelve cases had good prognosis to total resection and 2 recurrence due to subtotal or part resection. Conclusion cSFF is extremely rare, and it has some characteristics on MRI, final diagnosis should be depended on pathological and immunohistochemical examination. There are good prognoses in the patients with cSPT after the total removal of cSFT, but when the lesion could not be totally resected, radiotherapy should be considered, the curative effect of chemotherapy on cSFT is not definitive.