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1 岁患儿眶内孤立性纤维瘤:病例报告。

Solitary fibrous tumor of the orbit in a 1-year-old patient: a case report.

机构信息

Department of Ophthalmology and Visual Sciences, University of Louisville, Louisville, USA.

School of Medicine, University of Louisville, Louisville, Kentucky, USA.

出版信息

Orbit. 2024 Oct;43(5):615-618. doi: 10.1080/01676830.2023.2227695. Epub 2023 Jul 10.

DOI:10.1080/01676830.2023.2227695
PMID:37428951
Abstract

Solitary fibrous tumors (SFTs) are rare neoplasms of mesenchymal origin. While the mean age of presentation is 58 years old, we report the case of the youngest documented patient with an SFT of the orbit. A 13-month-old child was evaluated for eyelid asymmetry and then referred to the oculoplastic service. On examination, a soft tissue mass of the right inferomedial orbit was observed. MRI demonstrated a well-circumscribed, extraocular lesion in the inferomedial right orbit, potentially fibrous in nature. Excision was performed without complications. Pathologic examination demonstrated fibrous tissue proliferation with a staghorn vascular pattern, as well as benign fibrous cells with tapering nuclei and abundant pericellular reticulin. Immunohistochemistry (IHC) demonstrated that the cells stain diffusely positive for CD34 and vimentin. With the MRI findings, pathology, and IHC, the diagnosis was confirmed to be SFT. SFTs of the orbit, although rare, may occur in the pediatric population.

摘要

孤立性纤维瘤(SFT)是一种罕见的间叶来源的肿瘤。虽然其平均发病年龄为 58 岁,但我们报告了一例有记录以来最年轻的眶内 SFT 患者。一名 13 个月大的患儿因眼睑不对称就诊,随后被转诊至眼整形科。检查发现右眼内下象限有软组织肿块。MRI 显示右眼眶内下象限有一个界限清楚的眼外病变,可能为纤维性病变。手术切除无并发症。病理检查显示纤维组织增生,呈鹿角状血管模式,以及良性纤维细胞,细胞核逐渐变细,细胞周围有丰富的网状纤维。免疫组织化学(IHC)显示细胞弥漫性 CD34 和波形蛋白阳性。结合 MRI 结果、病理学和 IHC,诊断为 SFT。尽管罕见,但眶内 SFT 也可能发生于儿童。

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