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目的 :探讨血管肌纤维母细胞瘤 (AMFB)的临床病理特点及与侵袭性血管粘液瘤 (AA)的鉴别。方法 :对 5例AMFB和5例AA进行临床病理和免疫组化研究 ,对 3例AMFB进行电镜观察。结果 :AMFB位于外阴或腹股沟区 ,肿瘤边界清楚 ,大小 0 .8～ 4cm。光镜 :肿瘤细胞呈梭形上皮样、束状及巢状排列 ,常围绕小至中等大小的薄壁血管周围。肿瘤有细胞密集区和细胞分散区。免疫组化 :肿瘤细胞表达vimentin ,desmin ,ER和PR。电镜 :肿瘤细胞有的可见胞质内微丝和致密体结构 ,有原始细胞间连接。 2例AMFB随访 2年无复发。而 5例AA中有 2例复发。AA肿瘤细胞侵袭性生长 ,细胞核有明显的裂隙 ,厚壁血管周围常有肌样嗜伊红条索 ,有更丰富的粘液样基质。结论 :AMFB和AA是具有不同临床病理特点的肿瘤 ,可能均起源于血管周围向肌纤维母细胞分化的干细胞 ,两者的鉴别目前主要依据病理形态学特征。
Objective: To investigate the clinicopathological features of vascular myofibroblastic tumor (AMFB) and to distinguish it from invasive angiomyxoma (AA). Methods: Five cases of AMFB and five cases of AA were studied by clinical pathology and immunohistochemistry. Three cases of AMFB were observed by electron microscopy. Results: AMFB was located in the vulva or groin area. The tumor had a clear boundary with a size of 0.8 to 4 cm. Light microscopy: Tumor cells are spindle-like, bundle-like, and nest-like, often surrounding small to medium-sized thin-walled blood vessels. Tumors have cell-dense areas and cell dispersion areas. Immunohistochemistry: Tumor cells express vimentin, desmin, ER and PR. Electron microscopy: Some tumor cells can be seen within the cytoplasm of microfilament and dense structure, there is a connection between the original cells. Two patients with AMFB followed up for 2 years without recurrence. Of the 5 patients with AA, 2 had recurrence. Invasive growth of AA tumor cells, nucleus with obvious fissures, thick blood vessels often surrounded by muscle-like eosinophilic cords, there is a richer mucous-like matrix. Conclusion: AMFB and AA are tumors with different clinicopathological features. They may all originate from stem cells differentiated from perivascular to myofibroblasts. The discrimination between the two is currently based on pathological morphological features.