外阴血管肌纤维母细胞瘤2例病例报告  

Angiomyofibroblastoma of the vulva： report of 2 cases and review of the literature

作　　者：曹晓哲 陈维香[2] 童淑兰 费绍华 孙宏夫[2] 

机构地区：[1]珠海市第二人民医院病理科,519020 [2]上海市浦东新区公利医院病理科,200135

出　　处：《华夏医药》2007年第6期533-536,538,共5页Hua-xia Medicine

摘　　要：目的分析外阴血管肌纤维母细胞瘤（Angiomyofibroblastoma of the vulva，AMFB）的临床病理特征及鉴别诊断。方法对2例发生于外阴的AMFB进行病理形态观察及免疫组化标记，结合文献总结AMFB的临床病理特征及鉴别诊断。结果肿瘤境界清楚，直径〈5cm，镜下显示在粘液样和胶原纤维的背景中肿瘤细胞呈围血管排列的细胞密集区和细胞稀疏区交替分布的特征，肿瘤内血管丰富，大多为小一中等大小的薄壁血管。免疫表型：2例瘤细胞Vimentin均强阳性，1例瘤细胞Desmin（＋），1例Desmin（-）；肿瘤血管成分SMA，CD34，FⅧ均阳性，1例MSA．阳性，1例而阴性；CK，CD31，S-100均阴性。结论AMFB是一种少见的发生于女性生殖道的良性软组织肿瘤，可能起源于血管周围的具有多向分化潜能的干细胞，可向肌纤维母细胞方向分化。需与侵袭性的血管黏液瘤、富细胞性血管纤维瘤、浅表性的血管黏液瘤相鉴别。Purpose： To analyze the clinicopathological features and differential diagnosis of angiomyofibroblastoma of the vulva. Methods： Two cases of AMFB were examined by light microscope and immunohistochemical study. To discuss the clinicopathological features and differential diagnosis of AMFB with the help of an extensive revision of the literature. Results： Tumors were all circumscribed ,and 〈ScmlMicroscopically, the tumor were composed of spindle or polygonal cells which were cellularly or hypocellularly arranged with perivascular accentuation in a mucoid or fibrocollagenous background. The tumors contained numer- ous smallto medium-sized blood vessels, which were characteristically thin walled. Immunohistochemically, two cases tumor cells were all positive for vimentin, SMA, CD34 （＋）,FVIH （＋）,one case Desmin and muscle specific actin （MSA）, but not for cytokaractin,CD31,S-100 , another case Desmin and cytokaractin.Conclusion： AMF is a rare, benigh soft tissue tumor that occurs in the genital tract of adult women. The origin remains unclear, but it is suggested that an origin from a perivascular pluripotent stem cell that is cable of myofibroblastma differentiation. Angiomyo fibroblastoma should be differentiated from other neoplasms of the vulva such as aggressive angiomy^x oma,superficial angiomy^x oma and cellular angiofibroma.

关 键 词：外阴肿瘤 血管肌纤维母细胞瘤 鉴别 诊断 

分 类 号：R732.2[医药卫生—肿瘤]