血管肌纤维母细胞瘤临床病理分析

目的:观察并分析血管肌纤维母细胞瘤(angiomyofibroblastoma,AMF)的临床病理特点、影像学特征及治疗方法和预后。方法:回顾分析2015年至2019年诊断的5例AMF患者的临床资料,观察其临床特征、病理形态学特点及免疫组化结果等。结果:5例患者均为生育期女性,肿瘤位于外阴,临床以及实验室检查均缺乏特征性表现。镜下肿瘤边界清楚,并见大量的小到中等的薄壁血管。瘤组织由交替分布的细胞密集区和疏松区组成,密集区瘤细胞聚集于血管周围,成束或链状排列;疏松区瘤组织弥散于黏液背景中。免疫组化显示肿瘤细胞均表达Desmin、Vimentin、ER、PR,不表达CD31、CD34、S100、CKPan,1例表达SMA。结论:血管肌纤维母细胞瘤是种罕见的间叶组织肿瘤,好发于生育期的女性外阴,主要依据病理活检及免疫组化来明确诊断,首选手术切除以达到治疗效果。

Clinicopathological characteristics analysis of angiomyofibroblastoma

Objective:To investigate the clinicopathological characteristics,imaging features,treatments and prognosis of angiomyofibroblastoma(AMF).Methods:The clinical data of 5 cases of AMF diagnosed from 2015 to 2019 were retrospectively analyzed.The clinical features,pathomorphological characteristics and the results of immunohistochemistry were observed.Results:5 cases of AMF were all females in reproductive period.And the tumors were located in the vulva.The patients had no obvious clinical symptoms and no specificity in laboratory examination.Histologically,the tumors were all well circumscribed and had abundant small to medium sized thin-wall vessels.The tumors consisted of alternating loose and dense regions.The tumor cells in the dense area gathered around the vessels and arranged in cords or chains.The tumor cells in the loose area scattered in myxoid matrix.Immunohistochemically,the tumor cells were positive for Desmin,Vimentin,ER and PR,but negative for CD31,CD31,S100 and CKPan.Only one case was positive for SMA.Conclusion:AMF is a rare mesenchymal tumor that predominantly occurs in the vulval region of women in reproductive peroid.The accurate diagnosis is based on pathological biopsy and immunohistochemistry.Surgical excision is the main treatment method to make a fine prognosis.