Li Hongyun, Zhang Xuebei, Zhao Qingli, Wang Jin, Yang Xiaoqing, Huang Shengliang
Department of Urology, The First Affiliated Hospital of Shandong First Medical University and Shandong Provincial Qianfoshan Hospital, Jinan, Shandong 250014, P.R. China.
Nursing Department, Affiliated Eye Hospital of Shandong University of Traditional Chinese Medicine, Jinan, Shandong 250001, P.R. China.
Oncol Lett. 2023 Aug 4;26(3):409. doi: 10.3892/ol.2023.13995. eCollection 2023 Sep.
Epithelioid angiomyolipoma (EAML) of the kidney is an uncommon neoplasm with malignant potential. It can occur sporadically or be associated with tuberous sclerosis. EAML is a monotypic variant of angiomyolipoma (AML), which is classified as neoplasm of the perivascular epithelioid cell or perivascular epithelioid cell tumor. Due to its epithelioid nature and paucity of fat components, unlike classic AML, which has abundant adipose tissue with characteristic features on CT scans, it is difficult to distinguish EAML from renal cell carcinoma and fat-poor AML on CT or MRI preoperatively, which may lead to misdiagnosis and unnecessary nephrectomy. The present report describes two cases of renal EAML, which were successfully treated by laparoscopic surgery. Preoperative diagnosis had not been achieved until surgery was performed and histological analysis was accomplished. No local recurrence or distal metastasis was observed during follow-up. Although the differential diagnosis was challenging preoperatively, a diagnosis of EAML should be considered and surgical excision was the preferred treatment strategy for the patients with localized tumors.
肾上皮样血管平滑肌脂肪瘤(EAML)是一种具有恶性潜能的罕见肿瘤。它可散发性发生或与结节性硬化症相关。EAML是血管平滑肌脂肪瘤(AML)的一种单型变体,后者被归类为血管周上皮样细胞瘤或血管周上皮样细胞肿瘤。由于其上皮样性质且脂肪成分较少,与具有丰富脂肪组织且在CT扫描上有特征性表现的经典AML不同,术前在CT或MRI上很难将EAML与肾细胞癌及脂肪含量少的AML区分开来,这可能导致误诊及不必要的肾切除术。本报告描述了2例肾EAML,通过腹腔镜手术成功治疗。直到进行手术并完成组织学分析才实现术前诊断。随访期间未观察到局部复发或远处转移。尽管术前鉴别诊断具有挑战性,但对于局限性肿瘤患者,应考虑EAML的诊断,手术切除是首选的治疗策略。