外阴血管肌纤维母细胞瘤1例并文献复习

目的：分析外阴血管肌纤维母细胞瘤（Angiomyofibroblastoma，AMFB）的临床病理特征。方法：结合文献复习，对1例罕见的AFMB进行临床特点、病理形态及免疫组化研究，并探讨其鉴别诊断。结果：AMFB在肉眼上表现为境界清楚的结节；显微镜下，该瘤呈现为在富于水肿和富于薄壁血管的背景中见细胞密集区和细胞稀疏区交替存在，瘤细胞梭形、胖梭形或上皮样，围绕血管排列，间质中见散在肥大细胞和淋巴细胞浸润。免疫组化瘤细胞表达Vimentin、CD34、CD99、bcl-2和PR，不表达Desmin、S-100、ER、EMA、AE1／AE3以及SMA。结论：AMFB是一种罕见的良性肿瘤，手术切除后未见局部复发，罕见恶变，可经外科完全切除而治愈。主要应与外阴侵袭性血管粘液瘤鉴别。

One Case Report and Literatures Review of Vulva Angiomyofibroblastoma

To investigate the clinicopathological features of angiomyofibroblastoma(AMFB) of vulva. Methods: Clinical data, pathological features and immunolfistochemical data were amalyzed with review of literatures, and differential diagnosis was discussed in detail in one case of AMFB. Results: On gross examination, the tumor was well circumscribed, measuring 2.5cm in diameter. It was characterized by alternating hypercellular and hypocellular edematous zones in which abundant thin-walled blood vessels were irreguularly distributed. Spindle, plump spindle, and epithelioid tumour cells were aggregated around the blood vessels. Mast cells and lymphocytes were readily observed. The tumour cells expressed positively for vimentin, CD34, CD99, bcl-2 and PR, but negatively for desmin, S-100 protein, ER, EMA, cyterkeratin AE1/AE3 and alpha-SMA by immunohistochemical examinatim. Conclusions: AMFB is a benign tumour and should be distinguished from aggressive angiomyxoma.