摘要
目的总结血管肌纤维母细胞瘤(angiomyofibroblastoma,AMF)临床表现、影像学特征、组织病理表现和免疫组织化学特点、治疗方法和预后。方法回顾性分析12例AMF患者的临床资料。结果 12例均为女性,肿瘤位于外阴6例,阴道3例,宫颈2例,子宫体1例;临床表现、实验室检查及影像学表现缺乏特异性;12例肿瘤与周围组织界限相对清晰,可见较多薄壁血管,皮下纤维及肌纤维母细胞增生,瘤细胞呈梭形或星形,胞质透亮、无明显异型,核分裂像罕见;免疫组织化学示CD30、CD31、CD34、平滑肌肌动蛋白、MPO均(+),Desmin、雌激素受体、孕激素受体均为(),S-100、ALK均为(-),11例Ki-67(2%+),1例Ki-67(30%+);10例行单纯肿块切除术,1例行腹腔镜下全子宫+双侧输卵管切除术,1例化疗2个周期但肿瘤体积及累及范围未缩小;11例术后随访5a,生存率为90.9%。结论 AMF临床表现、实验室检查及影像学表现缺乏特异性,免疫组织化学示弥漫性阳性,治疗以手术切除为主,多数预后良好。
Objective To investigate the clinical,imaging,histopathological and immunohistochemical features as well as the treatment and prognosis of angiomyofibroblastoma（AMF）.Methods The clinical data of 12 patients with AMF were retrospectively analyzed.Results All these 12 patients with AMF were females,and the tumors were located at the vulva in 6,in the vagina in 3,at the cervix in 2,and at the uterine body in 1,lacking in clinical,laboratory and imaging specificities.The histopathological examination showed a clear boundary between the tumor and its surrounding tissue,more thin-walled blood vessels,and proliferated subcutaneous fibers and myofibroblasts.The tumor cells were in fusiform or star-shape,and the cytoplasm was translucent,with no obvious abnormality and rare mitosis.Immunohistochemistry showed positive CD30,CD31,CD34,smooth muscle actin and MPO in all patients,strong positive desmin,estrogen receptor and progesterone receptor in all,negative S-100 and ALK in all,Ki-67（2% ＋）in 11 patients and Ki-67（30%＋）in 1.Ten patients underwent simple mass resection,1 underwent laparoscopic hysterectomy plus bilateral salpingectomy,and 1 received chemotherapy for 2 cycles,with no shrinking of tumor size and its involved area.Eleven patients were followed up for 5 years,with the survival rate of 90.9%.Conclusion The clinical manifestations,laboratory and imaging features of AMF have no specificities,immunohistochemistry shows diffuse positive,and surgical resection is the main treatment method,with a fine prognosis.
作者
李晓燚
王悦
牟婧祎
LI Xiaoyi;WANG Yue;MOU Jingyi(Department of Gynecology and Obstetrics,Henan Provincial People＇s Hospital,People＇s Hosgital o f Zhenezhou University,Zhengzhou 450003,China)
出处
《中华实用诊断与治疗杂志》
2018年第11期1094-1095,共2页
Journal of Chinese Practical Diagnosis and Therapy
基金
河南省科技攻关计划项目(162102310174)
