上皮样血管平滑肌瘤(EAML),是血管平滑肌脂肪瘤(AML)的罕见亚型。EAML主要由单核或多核的上皮样细胞构成,特别是当肿瘤显示不典型的特征时很容易与有些肿瘤混淆。本文结合文献对其临床病理学特征、诊断和鉴别诊断、生物学行为、组织发生、流行病学和遗传学等进行讨论。 Epithelioid angiomyolipoma (EAML) is a rare subtype of angiomyolipoma (AML). EAML is mainly composed of mononuclear or multinucleated epithelioid cells, especially when tumors show atypical features that can easily be confused with some tumors. This article combines the literature on its clinical and pathological features, diagnosis and differential diagnosis, biological behavior, tissue development, epidemiology and genetics were discussed.