Hassan Mohammed, El-Hefnawy Ahmed S, Elshal Ahmed M, Mosbah Ahmed, El-Baz Mahmoud, Shaaban Attalah
Department of Urology, Mansoura Urology and Nephrology Center, Mansoura University, El-Gomhoria Street, P.O. Box: 35516, Mansoura, Egypt,
Int Urol Nephrol. 2014 Feb;46(2):317-22. doi: 10.1007/s11255-013-0510-3. Epub 2013 Aug 9.
To compare the clinical, pathological and oncological outcome of EAML with classic angiomyolipoma (AML).
A retrospective review of patients' files with a diagnosis of AML was conducted. Both classic and epithelioid AML were compared as regard the patients' demographics, mode of presentation, tumor features and follow-up data. The accuracy of preoperative radiological diagnosis of EAML and the postoperative pathological data were evaluated.
Between November 1988 and July 2012, 1,502 renal masses were treated. Forty-two patients (2.8 %) had renal AML of whom 12 patients (28.6 %) had epithelioid morphology. All the patients with EAML were symptomatic on presentation compared to 16.7 % asymptomatic patients with classic AML. Specific diagnosis of EAML was not suggested based on preoperative radiological evaluation. In one patient, there was a venous tumor extension into the right renal vein; another patient had a solitary pulmonary nodule. Pathological data of EAML patients showed wide areas of necrosis (4 patients), atypia (6 patients), increased mitotic figures (2 patients), nuclear anaplasia, enlarged nuclei and prominent nucleoli (3 patients), and multilobulated multinucleated giant cells (5 patients). At a median follow-up period of 12 and 9 months for EAML and classic AML groups, respectively, one patient with EAML had evidence of both local recurrence and distant metastasis 3 months following open radical nephrectomy.
Epithelioid AML is potentially aggressive and usually present as a symptomatic renal mass. It may be misdiagnosed as malignant renal mass on preoperative radiological evaluation.
比较上皮样血管平滑肌脂肪瘤（EAML）与经典血管平滑肌脂肪瘤（AML）的临床、病理及肿瘤学转归。
对诊断为AML的患者病历进行回顾性分析。比较经典型和上皮样AML患者的人口统计学资料、临床表现方式、肿瘤特征及随访数据。评估EAML术前影像学诊断的准确性及术后病理数据。
1988年11月至2012年7月期间，共治疗1502例肾肿块患者。42例（2.8%）患有肾AML，其中12例（28.6%）为上皮样形态。所有EAML患者就诊时均有症状，而经典AML患者中无症状者占16.7%。术前影像学评估未提示EAML的明确诊断。1例患者肿瘤静脉延伸至右肾静脉；另1例患者有孤立性肺结节。EAML患者的病理数据显示有大片坏死（4例）、异型性（6例）、核分裂象增多（2例）、核间变、核增大及核仁明显（3例），以及分叶状多核巨细胞（5例）。EAML组和经典AML组的中位随访期分别为12个月和9个月，1例EAML患者在开放性根治性肾切除术后3个月出现局部复发和远处转移。
上皮样AML具有潜在侵袭性，通常表现为有症状的肾肿块。术前影像学评估可能将其误诊为恶性肾肿块。
