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具有肌上皮癌分化的原发性皮肤双相性肉瘤样基底细胞癌:一种新变体。

Primary cutaneous biphasic sarcomatoid basal cell carcinoma with myoepithelial carcinoma differentiation: A new variant.

作者信息

Mestre-Alagarda Claudia, Nieto Gema, Terrádez Liria, Monteagudo Carlos

机构信息

Department of Pathology, Hospital Clínico Universitario, Universitat de Valencia, Valencia, Spain.

出版信息

J Cutan Pathol. 2019 Dec;46(12):949-953. doi: 10.1111/cup.13543. Epub 2019 Aug 1.

Abstract

Isolated cases of basal cell carcinoma (BCC) with partial myoepithelial component have been described. However, myoepithelial differentiation has not been described in sarcomatoid basal cell carcinomas, which usually show features resembling osteosarcoma, chondrosarcoma, or leiomyosarcoma. We report a case of an 87-year-old man with a forehead lesion that histologically showed a minor component of conventional nodular BCC in transition with a major biphasic sarcomatoid growth composed of invasive spindle-cell and epithelial-like components, the latter with a reticular pattern and scattered ductal structures. Both components showed cytological atypia and high mitotic rate (26/10HPF), with atypical mitotic figures. BER-EP4 immunostaining was exclusively found in the nodular BCC component whereas the sarcomatoid component revealed immunostaining for α-smooth muscle actin (SMA), muscle-specific actin (MSA), calponin, and p63 in both epithelial-like and spindle-cell populations. Focal immunoreactivity was observed in the epithelial component for S100 and glial fibrillary acidic protein (GFAP). Furthermore, EWSR1-PBX1 gene fusion was also detected. This is to our knowledge, the first fully documented case of biphasic sarcomatoid BCC with myoepithelial carcinoma differentiation.

摘要

已有关于伴有部分肌上皮成分的基底细胞癌(BCC)孤立病例的报道。然而,肌上皮分化在肉瘤样基底细胞癌中尚未见报道,肉瘤样基底细胞癌通常表现出类似骨肉瘤、软骨肉瘤或平滑肌肉瘤的特征。我们报告一例87岁男性前额病变病例,组织学检查显示有少量传统结节性BCC成分向主要的双相肉瘤样生长转变,后者由浸润性梭形细胞和上皮样成分组成,上皮样成分呈网状结构并散在分布导管结构。两种成分均显示细胞异型性和高有丝分裂率(26/10HPF),可见非典型有丝分裂象。BER-EP4免疫染色仅见于结节性BCC成分,而肉瘤样成分在上皮样细胞和平梭形细胞群体中均显示α-平滑肌肌动蛋白(SMA)、肌特异性肌动蛋白(MSA)、钙调蛋白和p63免疫染色阳性。上皮成分中观察到S100和胶质纤维酸性蛋白(GFAP)的局灶性免疫反应。此外,还检测到EWSR1-PBX1基因融合。据我们所知,这是首例有充分记录的具有肌上皮癌分化的双相肉瘤样BCC病例。

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