A Rare Case of Solitary Fibrous Tumor Involving the Nasolacrimal Duct System in a 12-Year-Old Female: A Case Report and Review of the Literature.

IF 1.2 4区 医学 Q3 OPHTHALMOLOGY
Nishita T Sheth, Modupe O Adetunji, Emily T Smith, Irene T Lee, Julie A Woodward, Thomas J Cummings, Christopher R Dermarkarian
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引用次数: 0

Abstract

The authors report the youngest case of solitary fibrous tumor (SFT) with extensive involvement of the nasolacrimal duct system and discuss current literature regarding this tumor type. A 12-year-old female presented with a 6-month history of an enlarging right medial orbit mass. CT orbits revealed a well-circumscribed, enhancing lesion adjacent to the right nasolacrimal system. Pathology confirmed SFT (1.2 × 1.1 × 1.1 cm) with spindle cell morphology and a mitotic rate of 5 per 10 high power fields. Immunohistochemistry (IHC) was positive for CD-34 and signal transducer and activator of transcription 6 and negative for S-100, consistent with SFT. Next-generation sequencing confirmed NGFI-A-binding protein 2::signal transducer and activator of transcription 6 gene fusion. To the authors' knowledge, only 17 cases of SFT involving the lacrimal sac have been reported, of which, the average age was 43.5 years. Notably, SFTs with a high mitotic rate carry a heightened risk of malignant transformation. Given this patient's mitotic rate of 5 per 10 high power fields, positive surgical margins, and young age, close follow-up is imperative.

一例罕见的 12 岁女性鼻泪管系统单发纤维性肿瘤病例:病例报告与文献综述。
我们报告一例最年轻的孤立性纤维性肿瘤(SFT),广泛累及鼻泪管系统,并讨论目前关于这种肿瘤类型的文献。一名12岁女性,6个月前出现右内侧眼眶肿大。CT眼眶显示右鼻泪系统附近边界清晰、强化病灶。病理证实为SFT (1.2 × 1.1 × 1.1 cm),呈梭形细胞形态,有丝分裂率为每10个高倍视场5个。免疫组化(IHC)检测CD-34和转录信号转导及激活因子6阳性,S-100阴性,与SFT一致。新一代测序证实ngfi - a结合蛋白2::信号转导和转录激活因子6基因融合。据我们所知,仅有17例涉及泪囊的SFT被报道,其中平均年龄为43.5岁。值得注意的是,有丝分裂率高的SFTs具有更高的恶性转化风险。考虑到患者的有丝分裂率为5 / 10的高倍视野,阳性手术切缘,以及年轻,密切随访是必要的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
2.50
自引率
10.00%
发文量
322
审稿时长
3-8 weeks
期刊介绍: Ophthalmic Plastic and Reconstructive Surgery features original articles and reviews on topics such as ptosis, eyelid reconstruction, orbital diagnosis and surgery, lacrimal problems, and eyelid malposition. Update reports on diagnostic techniques, surgical equipment and instrumentation, and medical therapies are included, as well as detailed analyses of recent research findings and their clinical applications.
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