肾脏上皮样血管平滑肌脂肪瘤的CT特征和预后分析

CT features and prognosis of renal epithelioid angiomyolipoma

目的分析。肾脏上皮样血管平滑肌脂肪瘤的CT特征和预后。方法回顾性分析连云港市第二人民医院和南京鼓楼医院泌尿外科2004年1月至2015年6月收治肾脏上皮样血管平滑肌脂肪瘤患者17例的临床资料。患者均接受CT检查，分析患者的一般资料、影像学特点、病理特点以及临床预后。结果9例患者行肾根治性切除术，8例行肾部分切除术。肿瘤直径1．2-12．5cm，平均为6．1cm。仅1例患者在CT中可以观察到脂肪成分。CT平扫9例肿瘤呈高密度、1例呈等密度、7例呈低密度。CT增强扫描1例轻度强化、6例中度强化、10例显著强化。其中8例均匀强化，9例强化不均。12例肿瘤HMB-45阳性，3例melanA阳性，2例HMB-45和melanA均阳性。术后随访2-126个月，平均28．5个月，1例患者出现局部复发和肺转移，1例患者出现肾上腺转移。结论肾脏上皮样血管平滑肌脂肪瘤CT表现不尽相同，直径10cm的肿瘤多数为实性，且CT平扫表现为高密度，当肿瘤直径10cm常伴有出血、坏死，且强化不均。与经典的肾脏血管平滑肌脂肪瘤不同，肾脏上皮样血管平滑肌脂肪瘤具有恶性潜能，可以出现局部复发和远处转移。

Objective To analyze the computed tomography （CT） features and clinical outcomes of renal epithelioid angiomyolipoma（EAML）. Methods From January 2004 to June 2015,17 patients with EAML in the Second People＇s Hospital of Lianyungang and Nanjing Drum Tower Hospital were included in the study. All patients underwent CT examination. The patients＇general data, imaging characteristics and pathologic features were determined by chart review. Results Nine patients underwent radical nephrectomy, and 8 patients underwent partial nephrectomy. The mean maximal tumor diameter was 6.1 cm （ ranged 1.2 - 12.5 cm）. The fat component of one lesion was detected by CT. On unenhanced CT, the intratumoral attenuations were hyperattenuating in 9 patients, isoattenuating in 1 patient and hypoattenuating in 7 patients. The contrast enhancement degree was mild in 1 patient, moderate in 6 patients and marked in 10 patients. The contrast enhancement pattern was homogeneous in 8 lesions and heterogeneous in 9 lesions. All patients were positive for melanoma（ 12 cases were positive for HMB -45,3 cases were positive for melan A, and 2 cases were positive for both）. The mean follow - up period was 28.5 months （ ranged 2 - 126 months）, and 15 patients were alive with no evidence of disease at the time of the last follow - up, 1 patient exhibited local recurrence and lung metastases,and another 1 patient developed distant metastasis. Conclusion Renal EAML has a range of imaging appearances. Our data suggested that the majority of the tumors （size 10 cm） were solid and had a tendency to be hyperattenuating on unenhanced CT images. Hemorrhaging or necrosis was observed in tumors with sizes≥ 10cm with heterogeneous enhancement. In contrast to classic AML, which is benign, EAML is potentially malignant and exhibits aggressive clinical features,including local recurrence and distant metastasis.