头皮低分化皮肤血管肉瘤的临床病理分析

Clinicopathologic study of 8 cases of poorly differentiated cutaneous angiosarcoma of scalp

目的 探讨头皮低分化皮肤血管肉瘤的临床免疫病理特征。方法 分析8例头皮低分化皮肤血管肉瘤患者的临床特点，观察其组织病理形态和CD31、CD34、第八因子相关抗原、波形蛋白、细胞角蛋白（AE1／AE3和CAM5．2）、上皮细胞膜抗原（EMA）、癌胚抗原（CEA）和S-100蛋白免疫组织化学染色。结果 患者平均发病年龄为69．0岁，男：女为5：3，临床早期常在头面部出现暗红斑，后期发生浸润性暗红斑块，均伴结节，偶发溃疡。组织病理检查示真皮广泛梭形细胞局部或广泛实性浸润，常见细胞异形，未见明确血管腔样结构。梭形细胞强阳性表达CD31、第八因子相关抗原和波形蛋白，弱阳性表达CD34、AE1／AE3和CAM5．2，而EMA、CEA和S-100蛋白阴性。结论 头面部出现淤斑样浸润性斑块和结节的老年患者，应及时做组织病理检查，CD31、CD34、第八因子相关抗原和波形蛋白免疫组织化学标记有助于明确诊断。

Objective To study the clinical and pathologic characteristics of poorly differentiated cutaneous angiosarcoma of scalp. Methods Eight cases of poorly differentiated cutaneous angiosarcoma of scalp were enrolled into this study. The clinical manifestations and histopathologic features were analyzed. Immunohistochemical study for CD31, CD34, factor Ⅷ-related antigen, vimentin, AE1/AE3, CAMS. 2, epithelial membrane antigen and carcinoembryonic antigen was performed. Results The mean age of the patients was 69 years. The male-to-female ratio was 5： 3. The tumor manifested clinically as bruise-like lesion in early phase, indurated erythematous plaque accompanied by nodules, ulcerations and bleeding in advanced phase. Histologically, the tumor was composed of solid sheets of undifferentiated spindle cells which were not easily recognizable as vascular in origin. Nuclear atypia was always present. The tumor cells in all of the 8 cases strongly expressed CD31, factor Ⅷ-related antigen and vimentin. Weak expression of CD34, AE1/AE3 and CAMS. 2 was noted in 2, 4 and 4 cases, respectively. The staining for epithelial membrane antigen, careinoembryonic antigen and S-100 was negative. Conclusions Angiosareoma needs to be excluded by histologic examination whenever bruise-like and erythematous lesions occurring on scalp skin of elderly patients. The endothelial origin of the tumor cells can be confirmed with immunostaining for CD31, CD34 and factor Ⅷ-related antigen.