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[眼眶孤立性纤维性肿瘤的临床病理观察]

[Clinicopathological observation of solitary fibrous tumor of the orbit].

作者信息

Lin Jin-yong, Sun Feng-yuan, Wang Yu-chuan, Yang Zhen-hai, Zhao Hong, Yang Li-hong

机构信息

Tianjin Eye Hospital, Tianjin Key Lab of Ophthalmology and Visual Science, Tianjin Eye Institute, the Ophthalmic College of Tianjin Medical University, Tianjin 300020, China.

出版信息

Zhonghua Yan Ke Za Zhi. 2012 Nov;48(11):976-80.

PMID:23302269
Abstract

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 (1 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.

摘要

目的

研究眼眶孤立性纤维瘤的临床病理特征。

方法

这是一项回顾性病例系列研究。对2003年1月至2011年12月在天津眼科医院接受治疗的8例眼眶孤立性纤维瘤的临床和病理特征进行回顾性分析。进行免疫组织化学染色,检测CD34、波形蛋白、S-100和平滑肌肌动蛋白(SMA)的表达。

结果

8例患者中,男性5例,女性3例;6例累及右侧眼眶,2例累及左侧眼眶。患者年龄30至60岁,平均年龄41岁;症状持续时间2个月至5年。肿瘤位于眼眶上半部分或颞上象限(5例)、泪囊及眼眶内侧(2例)或眼眶下半部分(1例)。临床特征主要包括上睑肿胀、单侧无痛性眼眶或泪囊肿块、眼球突出和复视。CT扫描通常显示边界清晰的中度或明显强化肿块。所有病例均行肿瘤完整切除。肿瘤大小为1.2 - 3.1 cm,呈圆形或不规则椭圆形肿块,有薄纤维包膜;3例肿瘤包膜不完整。显微镜下观察显示,肿瘤主要由梭形或椭圆形细胞组成,呈束状、席纹状、血管外皮细胞瘤样或“无特定模式”排列,有大量粗大胶原束,CD34和波形蛋白呈弥漫性阳性染色。5例患者术后随访6至86个月,这些患者均未发现局部复发迹象。

结论

眼眶孤立性纤维瘤是一种梭形细胞瘤,CD34和波形蛋白呈弥漫性阳性染色。该肿瘤通常边界清晰,有包膜。完整切除是首选治疗方法。应将该肿瘤与眼眶其他梭形细胞瘤相鉴别。

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