Yang Jian-Wei, Liang Cheng, Yang Li
Department of Urology, The Second Hospital of Lanzhou University, Lanzhou, China.
Clinical Center of Gansu Province for Nephron-urology, Lanzhou, China.
Kaohsiung J Med Sci. 2022 Oct;38(10):925-932. doi: 10.1002/kjm2.12586. Epub 2022 Sep 3.
Renal epithelioid angiomyolipoma (EAML) is a unique subtype of angiomyolipoma that contains a variety of cytoplasmic-rich, eosinophilic cytoplasm epithelioid cells in addition to mature adipocytes, hyaline thick-walled vessels, and smooth muscle-like spindle cells. In recent years, increasing evidence has shown that EAML is a potentially malignant tumor. Due to the lack of typical clinical manifestations and imaging features, it is difficult to diagnose before surgery, and the diagnosis mainly depends on postoperative histopathological examination. With the advancement of pathological diagnostic techniques, more EAML cases has been discovered, but clinicians still lack a comprehensive understanding of EAML. This review comprehensively describes some pathological and clinical features of EAML, with special attention to the pathogenesis and treatment of malignant EAML in order to assist with clinical diagnosis and treatment.
肾上皮样血管平滑肌脂肪瘤(EAML)是血管平滑肌脂肪瘤的一种独特亚型,除了成熟脂肪细胞、透明厚壁血管和平滑肌样梭形细胞外,还包含多种富含细胞质、嗜酸性细胞质的上皮样细胞。近年来,越来越多的证据表明EAML是一种潜在的恶性肿瘤。由于缺乏典型的临床表现和影像学特征,术前难以诊断,诊断主要依赖术后组织病理学检查。随着病理诊断技术的进步,发现了更多的EAML病例,但临床医生对EAML仍缺乏全面的了解。本文综述全面描述了EAML的一些病理和临床特征,特别关注恶性EAML的发病机制和治疗,以协助临床诊断和治疗。