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眼眶孤立性纤维性肿瘤:病例系列并文献复习

Orbital solitary fibrous tumor: a case series with review of the literature.

作者信息

Le Crystal P, Jones Scott, Valenzuela Alejandra A

机构信息

Department of Ophthalmology, Tulane University School of Medicine , New Orleans, Louisiana , USA.

出版信息

Orbit. 2014 Apr;33(2):145-51. doi: 10.3109/01676830.2013.853806. Epub 2013 Dec 2.

DOI:10.3109/01676830.2013.853806
PMID:24295271
Abstract

PURPOSE

To report the clinical features, patient demographics, management, and outcomes of a series of patients with orbital solitary fibrous tumors (OSFTs) and provide a review of the English literature describing this rare entity.

DESIGN

A review of patient demographics, clinical presentations, imaging, histopathology, surgical management, and outcomes were analyzed.

METHODS

A non-comparative retrospective chart review of the demographics, clinical presentations, imaging, histopathological features, management, and disease outcomes of patients presenting to a tertiary orbital center with a tissue diagnosis of OSFT between 2007-2012 was performed along with a review of the English-language literature.

RESULTS

Our study included four male patients, with a mean age of 48 years at referral. The most common presentations included a slowly growing mass, globe displacement, diplopia on extreme gazes, and/or facial disfiguration. All tumors were analyzed with histopathology and immunohistochemistry. Treatment involved the complete surgical excision of the lesion, obtaining clear margins. All patients remain alive and well with no evidence of recurrence after a minimum follow-up of 15 months (range 15 months-5 years).

CONCLUSION

SFTs should be considered when confronting a painless slowly growing orbital mass that induces globe displacement and/or facial deformity. Imaging will show a well-defined lesion that enhances with contrast. A careful initial surgical excision with clear margins is required for adequate local control of the tumor, avoiding recurrence and potential malignant transformation.

摘要

目的

报告一系列眼眶孤立性纤维瘤(OSFTs)患者的临床特征、患者人口统计学资料、治疗方法及预后,并对描述这一罕见疾病的英文文献进行综述。

设计

对患者人口统计学资料、临床表现、影像学检查、组织病理学检查、手术治疗及预后进行分析。

方法

对2007年至2012年间在一家三级眼眶中心就诊且经组织学诊断为OSFT的患者的人口统计学资料、临床表现、影像学检查、组织病理学特征、治疗方法及疾病预后进行非对照回顾性图表分析,并对英文文献进行综述。

结果

我们的研究纳入了4例男性患者,转诊时的平均年龄为48岁。最常见的表现包括缓慢生长的肿块、眼球移位、极度凝视时的复视和/或面部畸形。所有肿瘤均进行了组织病理学和免疫组织化学分析。治疗包括完整切除病变组织,切缘清晰。所有患者在至少随访15个月(范围15个月至5年)后均存活且情况良好,无复发迹象。

结论

当面对无痛性、缓慢生长且导致眼球移位和/或面部畸形的眼眶肿块时,应考虑孤立性纤维瘤。影像学检查将显示边界清晰的病变,增强扫描有强化。为充分控制肿瘤局部生长、避免复发和潜在的恶性转化,需要进行仔细的初次手术切除,切缘清晰。

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