Brightman Lori A, Demierre Marie-France, Byers H Randolph
Department of Dermatology, Boston University Medical School and Boston University Medical Center, Boston, MA 02118, USA.
J Cutan Pathol. 2006 Jan;33(1):38-42. doi: 10.1111/j.0303-6987.2006.00387.x.
Cutaneous epithelioid angiosarcoma is a type of cutaneous angiosarcoma and usually arise both on the head or neck of the elderly.
An 86-year-old male with an epithelioid angiosarcoma of the scalp that mimicked malignant melanoma.
A large irregular dark grey-blue plaque with an adjacent speckled tan nodule was suggestive of a primary cutaneous malignant melanoma with adjacent in-transit metastasis. Both had a well-circumscribed growth pattern and were composed of numerous large epithelioid cells with scattered severe atypia and mitoses. The tumor was positive for S-100 protein and vimentin and negative for low- and high-molecular weight cytokeratins. However, at high power, the epithelioid cells with severe atypia were negative for S-100 protein, and abundant large epithelioid macrophages were responsible for the S-100 protein positivity. The malignant tumor cells were negative for HMB-45, positive for CD31 and Factor VIII-related antigen, and focally positive for CD34. A focus of infiltrative, classical angiosarcoma with irregular vascular channels lined with plump, anaplastic endothelial cells was then found deep to the epithelioid tumor.
Macrophage-rich epithelioid angiosarcoma demonstrates abundant S-100 protein-positive epithelioid macrophages. This subset of epithelioid angiosarcoma may mimic malignant melanoma and may present as a pitfall in diagnosis.
皮肤上皮样血管肉瘤是皮肤血管肉瘤的一种类型,通常发生于老年人的头颈部。
一名86岁男性,头皮患有上皮样血管肉瘤,临床表现类似恶性黑色素瘤。
一个大的不规则深灰蓝色斑块,伴有一个相邻的斑点状棕褐色结节,提示原发性皮肤恶性黑色素瘤伴相邻的卫星转移。两者均呈边界清楚的生长方式,由大量大的上皮样细胞组成,伴有散在的重度异型性和核分裂象。肿瘤S-100蛋白和波形蛋白阳性,低分子量和高分子量细胞角蛋白阴性。然而,在高倍镜下,重度异型性的上皮样细胞S-100蛋白阴性,大量大的上皮样巨噬细胞导致S-100蛋白阳性。恶性肿瘤细胞HMB-45阴性,CD31和因子VIII相关抗原阳性,CD34局灶性阳性。在该上皮样肿瘤深部发现一个浸润性的经典血管肉瘤灶,有不规则的血管腔隙,内衬丰满的间变内皮细胞。
富含巨噬细胞的上皮样血管肉瘤可见大量S-100蛋白阳性的上皮样巨噬细胞。这种上皮样血管肉瘤亚型可能类似恶性黑色素瘤,可能是诊断中的一个陷阱。