眼眶孤立性纤维性肿瘤4例临床病理分析  

Orbital solitary fibrous tumors: a clinicopathological analysis of 4 cases

作　　者：黄彩虹[1] 丁永玲[1] 丁志燕[1] 刘爽[1] 

机构地区：[1]扬州市第一人民医院病理科,江苏扬州225002

出　　处：《诊断病理学杂志》2013年第6期359-362,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨眼眶孤立性纤维性肿瘤(SFT)的诊断、鉴别诊断及预后。方法结合文献对4例眼眶SFT的临床资料、病理学形态及免疫表型进行分析并随访。结果 4例眼眶SFT均为男性,平均年龄为46岁(37～57岁);左眼2例,右眼2例;主要临床表现为眼球突出及视力下降。光镜下SFT主要由梭形和类圆形细胞组成,呈无结构性生长,部分排列成束状、车辐状、席纹状等结构,间质有多少不等的胶原纤维沉积,密集细胞区和稀疏细胞区交替分布。免疫组化:肿瘤细胞CD34和vimentin均(+),2例bcl-2和CD99(+)。随访1例3年后再次复发,1例远处转移并死亡。结论眼眶SFT是一种少见的具有形态多样、无特殊组织构型及多少不等胶原纤维特征的间叶性肿瘤,对其确诊必需结合临床特征、病理形态及免疫表型,且需与眼眶其他软组织肿瘤相鉴别。SFT的生物学行为难以预料,肿瘤的形态与生物学行为不相关。肿瘤完整切除是预后良好的因素,对所有的SFT必须进行长期随访。Objective To investigate the diagnosis, differential diagnosis and prognosis of solitary fibrous tumors in orbit. Methods Four cases of SFT in the orbit were analyzed by clinical data analyses, HE staining and immunohistochemistry of EnVision technique, with review of the related literatures and follow-up. Results Four of the patients were male. The median age was 46 years （range, 37-57 years）. The two tumors were located in the right orbit and other two in the left orbit. The major clinical manifestation of the four SFT included proptosis, decreased vision. Microscopically, SFT consisted mainly of spindle and oval-shaped cells which showed patternless pattern. Some tumor cells arranged densely in fascicule, storiform or other pattern. Furthermore, a pattern showed interstitial collagen deposition. Hypercellular, dease areas alternated with hypocellular regions. Immunohistochemically, the over-expression of CD34, vimentin were observed in all cases. CD99 and bcl-2 were positive in two cases. Follow-up showed that 1 case had local recurrence, and another had metastasized distantly and died. Conclusions Orbital SFT is a rare orbital mesenchymal neoplasm that have morphological diversity, patternless pattern and interstitial collagen deposition. Its diagnosis should depend on clinical features, pathological and immunohistochemistry. It needs to be distinguished from other soft tissue tumors in orbit. The pathologic appearance of SFT cannot always predict clinical behaviors. Therefore, it is very important to conduct long-term follow-up to all of SFT after en bloc excision. If tumors could be removed completely, the patients may have a good prognosis.

关 键 词：眼眶 孤立性纤维性肿瘤 免疫组化 

分 类 号：R739.7[医药卫生—肿瘤]