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中国一家三级眼科医院眼眶孤立性纤维瘤患者的临床特征及预后

Clinical characteristics and prognosis of orbital solitary fibrous tumor in patients from a Chinese tertiary eye hospital.

作者信息

Ren Ming-Yu, Li Jing, Wu Yi-Xiang, Li Rui-Miao, Zhang Chi, Liu Li-Min, Wang Jing-Jing, Gao Yu

机构信息

Department of Orbital Disease and Ocular Tumor, Hebei Eye Hospital, Xingtai 054001, Hebei Province, China.

Intensive Care Unit, Xingtai Third Hospital, Xingtai 054001, Hebei Province, China.

出版信息

World J Clin Cases. 2022 Sep 26;10(27):9670-9679. doi: 10.12998/wjcc.v10.i27.9670.

Abstract

BACKGROUND

Solitary fibrous tumor (SFT) is predominant within the pleura but very rare in the orbit, which is why the diagnosis of orbital SFT poses challenges in clinical practice. Accordingly, an integrated approach that incorporates specific clinical features, histological, histopathological, and immunohistochemical (IHC) examinations, and molecular analyses is warranted.

AIM

To retrospectively explore the clinical and imaging characteristics, treatment, outcomes of a series of patients with orbital SFT.

METHODS

We conducted a retrospective review of a series of patients diagnosed with a histopathologic orbital SFT treated at a single institution. All data on demogra/phics, clinical characteristics, imaging, treatment, postoperative histopathological and IHC examinations, and prognosis were collected.

RESULTS

In total, 13 patients were enrolled, 7 (53.8%) of whom had the tumor located in the superomedial quadrant of the orbit. Computed tomography revealed a solitary ovoid lesion in 10 (76.9%) patients and irregular lesion in 3 (23.1%) patients. Magnetic resonance imaging results were as follows: On T1 weighted images, 3 (23.1%) patients had hypointense mixed signals, whereas 10 (76.9%) patients showed isointense mixed signals; on T2 weighted images (T2WI), 3 (23.1%), 4 (30.8%), and 6 (46.2%) patients exhibited hypointense mixed, isointense mixed, and hyperintense signals, respectively. Notably, 12 (92.3%) patients showed significant enhancement, whereas there were patchy slightly enhanced areas in the tumor. All patients were treated by surgery. IHC analysis demonstrated that the tumor cells were immunoreactive for CD34, CD99, STAT-6, and vimentin in all patients. The lesions showed Ki-67 positivity < 5% in 1 (7.7) patient, 5%-10% in 10 (76.9%), and > 10% in 2 (15.4%). Two (15.4%) patients exhibited tumor recurrence.

CONCLUSION

The clinical manifestations and radiologic characteristics of orbital SFT are diverse and not specific. Accurate diagnosis and treatment require detailed radiological and histopathological/IHC evaluation.

摘要

背景

孤立性纤维瘤(SFT)主要发生于胸膜,但在眼眶中极为罕见,这就是为什么眼眶SFT的诊断在临床实践中具有挑战性。因此,需要一种综合方法,结合特定的临床特征、组织学、组织病理学和免疫组化(IHC)检查以及分子分析。

目的

回顾性探讨一系列眼眶SFT患者的临床和影像学特征、治疗方法及预后。

方法

我们对在单一机构接受组织病理学诊断为眼眶SFT的一系列患者进行了回顾性研究。收集了所有关于人口统计学、临床特征、影像学、治疗、术后组织病理学和IHC检查以及预后的数据。

结果

共纳入13例患者,其中7例(53.8%)肿瘤位于眼眶的内上象限。计算机断层扫描显示,10例(76.9%)患者为孤立性卵圆形病变,3例(23.1%)患者为不规则病变。磁共振成像结果如下:在T1加权图像上,3例(23.1%)患者表现为低信号混合信号,而10例(76.9%)患者表现为等信号混合信号;在T2加权图像(T2WI)上,3例(23.1%)、4例(30.8%)和6例(46.2%)患者分别表现为低信号混合、等信号混合和高信号。值得注意的是,12例(92.3%)患者表现出明显强化,而肿瘤内有散在的轻度强化区域。所有患者均接受了手术治疗。免疫组化分析表明,所有患者的肿瘤细胞对CD34、CD99、STAT-6和波形蛋白均呈免疫反应性。1例(7.7%)患者病变的Ki-67阳性率<5%,10例(76.9%)为5%-10%,2例(15.4%)>10%。2例(15.4%)患者出现肿瘤复发。

结论

眼眶SFT的临床表现和影像学特征多样且不具有特异性。准确的诊断和治疗需要详细的影像学和组织病理学/免疫组化评估。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5af2/9516925/32c9daabfe93/WJCC-10-9670-g001.jpg

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