眼眶孤立性纤维瘤的临床病理学观察  

Clinicopathological observation of solitary fibrous tumor of the orbit

作　　者：林锦镛[1] 孙丰源[2] 王玉川[1] 杨振海[1] 赵红[1] 杨丽红[1] 

机构地区：[1]天津市眼科医院天津市眼科学与视觉科学重点实验室天津市眼科研究所天津医科大学眼科临床学院,300020 [2]天津市第一中心医院眼科

出　　处：《中华眼科杂志》2012年第11期976-980,共5页Chinese Journal of Ophthalmology

摘　　要：目的分析眼眶孤立性纤维瘤的临床病理学特点。方法回顾性系列病例研究。对天津市眼科医院2003年1月至2011年12月收治的8例眼眶孤立性纤维瘤患者的临床病理学资料进行总结，分析其临床和病理学特点。结果8例患者中，男性5例，女性3例；肿瘤位于右眼眶6例，左眼眶2例；发病年龄30～60岁，平均41岁；病程2个月～5年；5例肿瘤位于眼眶外上方或上方，2例肿瘤位于眼眶内侧泪囊周围，1例肿瘤位于眼眶下方。临床主要表现为上眼睑肿胀、眼眶内或泪囊部肿物、眼球突出、复视，无明显疼痛。CT检查结果显示，肿瘤呈中高密度的实质性占位影，密度比较均匀，边界清楚。8例肿瘤均行肿瘤完整摘除术。肿瘤大体呈圆形或不规则的椭圆形。最大直径为1．2—3．1cm，表面有很薄的纤维膜包绕，其中3例包膜不完整。肿瘤主要由梭形或卵圆形肿瘤细胞组成，肿瘤细胞无明显异型性，排列成席纹状、束状、血管外皮瘤样或呈无模式性生长方式，肿瘤细胞间穿插粗大的胶原纤维和大量树枝状或鹿角状的血管。免疫组织化学染色结果显示，多数肿瘤细胞对CD34和波形蛋白抗体呈弥漫性阳性表达。术后5例获得随访，均无肿瘤复发。结论眼眶孤立性纤维瘤是一种梭形细胞性肿瘤，肿瘤细胞对CD34和波形蛋白抗体呈弥漫性阳性表达。多数肿瘤边界清楚，有很薄的纤维性包膜，完整切除肿物是首选治疗方法。诊断中应注意与眼眶其他梭形细胞性肿瘤相鉴别。Objective To study the clinicopathological characteristics of orbital solitary fibrous tumor. Methods It was a retrospective case series study. The clinical and pathological characteristics of 8 cases of orbital solitary fibrous tumor that were treated in Tianjin Eye Hospital from January 2003 to December 2011 were reviewed and analyzed. Immunohistochemical staining was performed, investigating the expression of CD34, vimentin, S-100 and SMA. Results In the 8 cases, 5 cases were male and 3 cases were female; 6 cases involved the right orbit and 2 cases involved the left orbit. Patients＇ ages ranged from 30 to 60 years, and the mean age was 41 years ~ duration of symptoms ranged from 2 months to 5 years. The locations of the tumor were in the superior or superotemporal orbit （5 cases） , lacrimal sac and medial orbit （2 cases） , or inferior orbit （ l case）. The clinical features mainly included the swelling of the upper lid, unilateral painless orbital or lacrimal sac mass, proptosis and diplopia. CT scan usually revealed a moderately or intensely enhanced mass that was well-circumscribed. Complete tumor removal was performed in all cases. The tumor sizes ranged from 1.2 - 3.1 cm ＇and appeared as a round or irregular oval mass with a thin fibrous capsule; incomplete capsule was found in 3 tumors. Microscopic observation revealed that the tumor was composed mainly of spindle or oval cells in a fascicular, storiform, hemangiopericytoma-like or a ＂patternless＂ pattern, with numerous thick bands of collagen that showed diffuse positive staining for CD34 and vimentin. Five cases had been followed-up from 6 to 86 months postoperatively, no evidence of local recurrence had been found in these patients. Conclusions Orbital solitary fibrous tumor is a spindle cell tumor with diffuse positive staining for CD34 and vimentin. This tumor usually is well circumscribed and encapsulated. Complete excision is the preferred treatment. The tumor should be distinguished from other spindle cell tumors in the orbit.

关 键 词：眶肿瘤 孤立性纤维瘤 病理学 临床 

分 类 号：R739.72[医药卫生—肿瘤]