矢状窦旁孤立性纤维瘤一例并文献复习

Report and review of the literatures of a case of parasagittal solitary fibrous tumor

目的总结分析中枢神经系统孤立性纤维瘤(cSFT)的诊断与手术治疗。方法回顾分析2011年3月2日中山大学附属第三医院神经外科收治的1例矢状窦旁孤立性纤维瘤的临床、磁共振成像(MRI)、病理特征及手术治疗效果,并作文献复习。患者因进行性左下肢乏力1年余入院,MRI显示右侧额顶部大脑镰旁巨大占位,与硬脑膜相连,T1WI呈等稍低信号,T2WI呈等稍高信号,增强扫描明显均匀强化,并侵犯上矢状窦。结果对患者右侧额顶部马蹄形切口,骨窗跨过中线,术中见肿瘤与脑组织边界清楚,血供非常丰富,与硬脑膜、上矢状窦及大脑镰黏连紧密,并侵犯上矢状窦,大小约6.0 cm×6.0 cm×6.0 cm,不规则,呈灰红色,质韧。分块完全切除肿瘤,术后未见并发症。病理显示肿瘤由梭形和短梭形细胞弥漫增生构成,呈分叶状,部分区域细胞密集,部分区域细胞稀疏,稀疏区见较多胶原纤维,其内血管有玻璃样变性,密集区灶性区域有血管外皮瘤样结构,细胞异型性不明显,核分裂少见,免疫组化vimentin(+)、CD34(+),S100(-),EMA(-);随访1年,左下肢肌力基本恢复,未见复发。结论 MRI影像上cSFT很难与颅内脑膜瘤鉴别;免疫组化对cSFT的诊断有极其重要意义,cSFT呈vimentin、CD34、CD99及bcl-2阳性,而S100、EMA阴性;手术完全切除是治疗cSFT的最佳方案,是否全切是最重要的预后因素;应做长期密切随访。

Objective To study the diagnosis and treatment of solitary brous tumor of the central nervous system( cSFT). Methods The clinical features,magnetic resonance imaging( MRI),pathological characteristics and surgical treatment of a case of parasagittal solitary fibrous tumor of 3rd Affiliated Hospital of SUN YAT-SEN University in March 3 rd 2011 were analyzed and studied retrospectively. The patient was progressive left leg weak more than 1 year. MRI showed that a huge lesion was in the right frontal of parietal lobe and was attached to the dural. T1WI showed equal slightly lower signal,T2WI showed slightly higher signal. It was significantly enhanced homogeneously,and invaded the superior sagittal sinus. Results At operation,the margin between the tumor and brain tissue was clear. The tumor appeared very rich blood supply. It attached to the dura,superior sagittal sinus,falx cerebri and invaded the superior sagittal sinus. The tumor appeared to be irregular,tough,gray-red and about 6. 0 cm × 6. 0 cm × 6. 0 cm. Block and total removal of the tumor were performed. No complications were observed. Pathology showed that the tumor was lobulated and was formed by the diffuse proliferative spindle and short spindle cell. It had hypercellular and hypocelluar areas. More collagen fibers and the blood vessels with hyaline degeneration were observed within hypocelluar areas. Hemangiopericytoma-like structure were observed within hypercellular area. Atypia and mitotic were difficult to observed. Immunohistochemistry showed vimentin( +),CD34( +),S100(-),EMA(-). With followed up one year,the left leg muscle strength was recovered and no recurrence were observed. Conclusions cSFT is difficult to be differentiated from intracranial meningiomas by magnetic resonance imaging. Immunohistochemical diagnosis of cSFT is of vital importance. cSFT shows positive for vimentin,CD34,CD99,bcl-2 and negative for S100,EMA. Radical surgical excision is the best treatment plan and the most important prognostic factor. Long-term close follow-up remain mandatory for all cSFT.