血管肌纤维母细胞瘤16例临床病理分析

目的探讨血管肌纤维母细胞瘤（angiomyofibroblastoma，AMF）的临床病理、免疫组化特征、组织来源以及鉴别诊断。方法通过16例AMF的临床表现、病理形态和免疫组化研究，并结合文献复习，总结AMF的临床病理、免疫组化特征及鉴别诊断。结果16例AMF，大体境界清楚，无包膜，可见一层假纤维膜包绕；组织学上呈疏松水肿样，丰富薄壁海绵样血管，肿瘤细胞多样性，梭形及上皮样细胞多见，常围绕血管排列呈疏密交替分布特征。免疫组化结果：瘤细胞desmin及vimentin呈弥漫强阳性表达，actin、CD34、ER和PR染色稍弱、呈灶状分布，而S-100蛋白、NF和CK均阴性。结论AMF临床上多无明显症状；发病部位以女性外阴及盆腔最多见，但全身多处部位也可见；其生物学行为及组织来源尚无定论；术前很难确定诊断AMF，需与侵袭性血管黏液瘤、富于细胞性血管纤维瘤等相鉴别。

Angiomyofibroblastoma: A clinicopathologic analysis of 16 cases

Purpose To study the clinicopathologic characteristics and immunohistochemical features of angiomyofibroblastoma (AMF) and its histogenesis, as well as differential diagnosis. Methods With review of the literatures, histopathology and immunohistochemistry were used to investigate the clinical appearance, pathological feature and differential diagnosis in 16 cases of angiomyofibroblastoma. Results Angiomyofibroblastoma was well circumscribed, and characterized by alternating hypercellular and hypocellular edematous zones with numerous blood vessels. Tumor cells were spindled or oval, concentrated around the blood vessels. Immunohistochemically, the tumor cells were strong positive for vimentin and desmin, only local positive for CD34, ER and PR, but all tumor cells were negative for S-100 protein, NF and CK. Conclusions AMF is a soft tissue neoplasm that occurs all over the body, principally in the vulval region of the reproductive age woman. The histophathologic origin and prognosis is still uncertain. It needs to make differential diagnoses from several tumors, such as aggressive angiomyxoma and cellular angiofibroma.