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伪装成皮样囊肿的浅表孤立性纤维瘤:一例报告

Superficial solitary fibrous tumor masquerading as a dermoid cyst: a case report.

作者信息

Foss Michael G, Dunn Charles, Marks Etan, Nathoo Rajiv

机构信息

Department of Dermatology, Kansas City University Graduate Medical Education Consortium/Advanced Dermatology and Cosmetic Surgery, Orlando, FL, USA.

出版信息

AME Case Rep. 2022 Oct 30;6:34. doi: 10.21037/acr-22-17. eCollection 2022.

Abstract

BACKGROUND

Solitary fibrous tumors (SFTs) are relatively rare spindle cell neoplasms uncommonly seen in dermatology practice. Initially discovered as a pleural tumor, SFTs have also been found in extra-pleural sites including the skin and soft tissues. When arising within the dermis or subcutis they are termed superficial SFTs, where they often present as solitary, unilateral, slow growing superficial masses. Histologically, they are composed of spindle cells arranged in a "patternless" pattern with hemangiopericytoma-like vessels dispersed throughout. Historically, CD34, CD99 and Bcl-2 immunohistochemical (IHC) stains were used to differentiate SFTs from other spindle cell neoplasms, however these markers are not entirely specific. Recent discovery of a disease defining NGFI-A binding protein 2 ()-signal transducer and activator of transcription 6 () fusion gene has led to the use of STAT6 IHC staining to help verify the diagnosis of SFTs, particularly in unexpected sites.

CASE DESCRIPTION

We report a case of a 23-year-old woman with a slowly growing lateral supra-orbital mass, clinically concerning for a dermoid cyst, which was subsequently discovered to be a SFT on pathologic examination, with the diagnosis being verified by STAT6 immunostaining.

CONCLUSIONS

SFTs are rarely encountered in dermatologic practice, however, must be kept on the differential of subcutaneous nodules, including those occurring in young adults. Due to the rarity of these tumors in clinical practice, a proposed algorithm for the approach to management of SFTs is included, guided by a validated, histology-driven, metastatic risk assessment tool, to help guide other clinicians confronted by these tumors.

摘要

背景

孤立性纤维瘤(SFTs)是相对罕见的梭形细胞瘤,在皮肤科临床实践中并不常见。SFTs最初作为胸膜肿瘤被发现,也已在包括皮肤和软组织在内的胸膜外部位被发现。当发生于真皮或皮下组织时,它们被称为浅表性SFTs,通常表现为孤立、单侧、生长缓慢的浅表肿块。组织学上,它们由梭形细胞组成,呈“无模式”排列,其间散在分布着类似血管外皮细胞瘤的血管。从历史上看,CD34、CD99和Bcl-2免疫组化(IHC)染色曾用于将SFTs与其他梭形细胞瘤相鉴别,然而这些标志物并非完全特异。最近发现的一种疾病定义性的NGFI-A结合蛋白2()-信号转导和转录激活因子6()融合基因,使得STAT6 IHC染色被用于帮助验证SFTs的诊断,尤其是在意外部位。

病例描述

我们报告一例23岁女性,其眶上外侧有一生长缓慢的肿块,临床怀疑为皮样囊肿,病理检查后发现是SFT,并通过STAT6免疫染色得以确诊。

结论

SFTs在皮肤科临床实践中很少见,但必须列入皮下结节的鉴别诊断范围,包括发生在年轻人中的结节。由于这些肿瘤在临床实践中罕见,本文纳入了一种基于经过验证的、组织学驱动的转移风险评估工具所提出的SFTs管理方法算法,以帮助指导其他面对这些肿瘤的临床医生。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/43e2/9634459/4504aaff6eb2/acr-06-22-17-f1.jpg

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