中枢神经系统孤立性纤维瘤的诊断和治疗

Diagnosis and treatment of solitary fibrous tumor of the central nervous system

目的探讨中枢神经系统孤立性纤维瘤（cSFT）这一罕见疾病的临床特征、诊断要点及治疗原则。方法收集2009年至2015年经病理证实cSFT的患者22例，对所有患者术前症状，影像学表现，病理学特征，治疗方案及预后等资料进行回顾性分析。结果本组患者中男性10例，女性12例，患者年龄自23～70岁，中位年龄44岁；11例位于幕上，6位于幕下，5例位于椎管内；MRI提示19例病灶为边界清楚的实性占住，其T1WI呈等低信号，T2WI呈高低混杂信号，另外3例含囊性成分，其T1、T2相信号明显不均；所有肿瘤平扫T2低信号区域增强效应明显；19例患者获全切，除2例老年患者失访外，均无复发，3例行次全切者均行伽马刀辅助放疗，其中两例复发。组织学检查提示肿瘤良性者18例，恶性4例。免疫组化CD34、Bcl-2、CD99、Vimentin均阳性，Des、S-100均阴性。结论cSFT在中枢神经系统及其罕见且易复发，其影像学表现具一定特点，最终诊断仍靠病理活检及免疫组化。cSFT首选手术治疗，若能全切则总体预后较好，肿瘤未全切者术后放射治疗效果不确定，所有患者术后需密切随访。

Objective To study the clinical characteristics, diagnostic findings and therapeutic management of solitary fibrous tumor of the central nerous system （cSFT）. Methods Between 2009 and 2015, 22 patients harboring cSFTs were treated at our institution. We retrospectively reviewed clinical presentation, imaging characteristics, pathological features, treatment strategies and prognosis with associated clinical records. Results The 22 patients included 10 men and 12 women with a median age of 44 years. The locations were 11 cases （50%） at supratentorial, 6 cases （27%） at posterior fossa and 5 cases （23%） at spinal. 19 lesions were found as solitary and well-defined mass and were heterogeneous with areas of hypo to isointensity on TlWI and hypo to hyperintensity on T2WI. The other 3 were cystic and showed mixed signal intensity on T1 and T2-weighted imaging. All of the areas showed hypointensive signal on T2WI revealed marked enhancement. Gross total resections （GTR） and subtotal resections （STR） followed by gama knife radiosurgeries （GKRS） were achieved in 19 and 3 cases, respectively. No tumor recurrence occurred in these GTRs,excluding 2 elder cases out of follow-ups. 2 STR recurred. Pathologically, 18 and 4 cases were classified as benign and malignant, respectively. CD34, CD99 and vimentin were 100% positive, but EMA and S-100 100% negative. Conclusion cSFT is a rare mesenchymal tumor with a propensity to recur. It has some characteristics on imaging findings, while the final diagnosis depends mainly on histopathology and immunohistochemistry. Surgical resection is the preferred treatment strategy for cSFT and it should has a good prognosis when GTRs were performed. The curative effect of GKRS is not definitive. Regular follow-ups are mandatory for all cSFT.