Cutaneous Clear Cell Sarcoma in the Genital Region: A Report of Two Cases at an Unusual Location.

IF 1.1 4区 医学 Q3 DERMATOLOGY
Zhongqin Meng, Mary Elizabeth Reed Shenk, Dean Holliday, Dina V Hunter, Pelin Sagut, Jessica A Forcucci, Zhihua Zhao, Wencai Li, Dirk M Elston
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引用次数: 0

Abstract

Clear cell sarcoma (CCS) is a rare sarcoma that typically arises in the deep soft tissue of the extremities. We describe two patients with molecularly confirmed CCS in genital skin. Patient 1 was a 22-year-old woman with a vulvar mass, and Patient 2 was a 25-year-old man with a scrotal plaque. Histopathological examination in each case revealed a cellular tumor distributed in nests and sheets, composed of epithelioid and spindled cells. The tumor cells exhibited clear and eosinophilic cytoplasm, vesicular nuclei, and prominent nucleoli. Immunohistochemical analysis demonstrated that both tumors expressed Sox10 and HMB45. FISH testing revealed EWSR1 rearrangements in both cases, and further NGS analysis in Case 1 confirmed the presence of an EWSR1-ATF1 fusion. To date, only 3 cases of CCS have been reported involving the genital region, all originating in deep soft tissues. Our report expands the known anatomical sites of cutaneous clear cell sarcoma involvement.

皮肤透明细胞肉瘤在生殖器区域:报告两例在一个不寻常的位置。
透明细胞肉瘤(CCS)是一种罕见的肉瘤,通常发生在四肢的深层软组织。我们描述了两例分子证实的生殖器皮肤CCS患者。患者1为22岁女性外阴肿块,患者2为25岁男性阴囊斑块。组织病理学检查均显示细胞肿瘤呈巢状和片状分布,由上皮样细胞和梭形细胞组成。肿瘤细胞细胞质清晰,嗜酸性,胞核呈泡状,核仁明显。免疫组化分析显示两种肿瘤均表达Sox10和HMB45。FISH检测显示,这两例病例中都出现了EWSR1重排,病例1的进一步NGS分析证实了EWSR1- atf1融合的存在。迄今为止,仅报告了3例涉及生殖器区域的CCS病例,均起源于深部软组织。我们的报告扩大了已知的皮肤透明细胞肉瘤累及的解剖部位。
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来源期刊
CiteScore
3.20
自引率
5.90%
发文量
174
审稿时长
3-8 weeks
期刊介绍: Journal of Cutaneous Pathology publishes manuscripts broadly relevant to diseases of the skin and mucosae, with the aims of advancing scientific knowledge regarding dermatopathology and enhancing the communication between clinical practitioners and research scientists. Original scientific manuscripts on diagnostic and experimental cutaneous pathology are especially desirable. Timely, pertinent review articles also will be given high priority. Manuscripts based on light, fluorescence, and electron microscopy, histochemistry, immunology, molecular biology, and genetics, as well as allied sciences, are all welcome, provided their principal focus is on cutaneous pathology. Publication time will be kept as short as possible, ensuring that articles will be quickly available to all interested in this speciality.
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