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非典型纤维黄色瘤:病例系列及文献综述

Atypical Fibroxanthoma: A case series and review of literature.

作者信息

Mahalingam Sridhayan, Shah Aadarsh, Stewart Andrew

机构信息

Epsom and St Helier University Hospitals NHS Trust, Wrythe Lane, Carshalton SM5 1AA, United Kingdom.

North Middlesex University Hospital, Sterling Way, London N18 1QX, United Kingdom.

出版信息

Auris Nasus Larynx. 2015 Dec;42(6):469-71. doi: 10.1016/j.anl.2015.04.001. Epub 2015 Apr 22.

DOI:10.1016/j.anl.2015.04.001
PMID:25912604
Abstract

INTRO/OBJECTIVE: Atypical Fibroxanthoma (AFX) is a rare cutaneous neoplasm arising from myofibroblast or fibroblast-like cells that predominantly affects the head and neck region. It commonly mimics more invasive neoplasms and is a diagnostic challenge to clinicians. The aim of this study was to develop a better understanding of AFX, focusing on recent developments in diagnosis and management.

METHODS

A retrospective case series and review of recent literature were carried out.

RESULTS

Over a 17-year period, seven cases were identified (six male, mean age at presentation was 75.9 years). Two patients underwent complete excision and five patients had curettage and cauterisation. Two patients developed local recurrence but none demonstrated signs suggestive of metastatic spread. Histologically all seven lesions displayed a spindle cell pattern. Where performed, immunohistochemical staining was positive for Vimentin, CD10, CD68 and actin, and negative for CAM 5.2, CD34, Melan-A, S100 protein, HMB45, Cytokeratin A1/A3.

CONCLUSION

Our patient demographics, histopathology and immunohistochemistry are comparable to previous studies. Although advances have been made in immunohistochemical analysis, we are yet to discover a specific diagnostic immunostain for AFX. Clinical findings should therefore be correlated with histology and a panel of immunohistochemical stains should be used. Given the potential for recurrence or metastases, Moh's Micrographic Surgery with regular follow-up may be the preferred management.

摘要

引言/目的:非典型纤维黄色瘤(AFX)是一种罕见的皮肤肿瘤,由肌成纤维细胞或成纤维细胞样细胞产生,主要累及头颈部区域。它通常类似更具侵袭性的肿瘤,对临床医生来说是一个诊断挑战。本研究的目的是更好地了解AFX,重点关注诊断和治疗的最新进展。

方法

进行了一项回顾性病例系列研究并对近期文献进行了综述。

结果

在17年的时间里,共确定了7例病例(6例男性,就诊时的平均年龄为75.9岁)。2例患者接受了完整切除,5例患者进行了刮除和烧灼治疗。2例患者出现局部复发,但均未表现出转移扩散的迹象。组织学上,所有7个病变均显示梭形细胞模式。在进行免疫组化染色的病例中,波形蛋白、CD10、CD68和肌动蛋白呈阳性,而CAM 5.2、CD34、Melan-A、S100蛋白、HMB45、细胞角蛋白A1/A3呈阴性。

结论

我们的患者人口统计学、组织病理学和免疫组化结果与先前的研究相当。尽管免疫组化分析取得了进展,但我们尚未发现AFX的特异性诊断免疫染色。因此,临床发现应与组织学相关联,并应使用一组免疫组化染色。鉴于存在复发或转移的可能性,Moh's显微外科手术并定期随访可能是首选的治疗方法。

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