Department of Urology and Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 2010 Sep;51(5):728-34. doi: 10.3349/ymj.2010.51.5.728.
The purpose of this study is to set guidelines for the management of renal angiomyolipoma (AML), clinical prognosis according to tumor size, in association with tuberous sclerosis complex (TSC), multiplicity, radiographic finding, and treatment modality.
Between March 1998 and October 2008, 129 out of 254 patients with AML who underwent surgical intervention or angioembolization were enrolled. Diagnosis of AML was determined by the presence of a low attenuated component on CT imaging or by pathological confirmation. Indications of treatment were intractable pain, hematuria, suspicion of malignancy, large tumor size, spontaneous rupture, and radiographically equivocal tumors in which a differential diagnosis was needed to rule out malignancy. Parameters including age, sex, tumor size, multiplicity, radiographic characteristics, association with TSC, and treatment modality were reviewed.
Age at presentation was 50.6 years and mean tumor size was 3.5 cm. Presentation symptoms were flank pain, hematuria, spontaneous rupture, and fatigue. 97 (75.2%) patients were incidentally discovered. 100 (77.5%) were females. 68 (52.7%) underwent nephron-sparing surgery (NSS), 35 (27.1%) radical nephrectomy, and 26 (20.2%) angioembolization. TSC was accompanied in 12 (9.3%) patients. No patient developed renal function impairment during the mean follow-up period of 64.8 months. Patients with TSC presented at a younger age, along with larger, bilateral, and multiple lesions.
Significant differences in clinical manifestations and treatment outcomes were noted in respect to tumor characteristics, association with TSC, and treatment modality. Considering the benign nature of AML, these parameters ought to be considered when deciding upon active surveillance or prophylactic intervention.
本研究旨在为肾血管平滑肌脂肪瘤(AML)的管理制定指南,根据肿瘤大小、与结节性硬化症(TSC)的关系、多发性、影像学表现和治疗方式来预测其临床预后。
1998 年 3 月至 2008 年 10 月,我们对 254 例接受手术干预或血管栓塞治疗的 AML 患者中的 129 例进行了回顾性分析。AML 的诊断依据是 CT 成像上存在低衰减成分或病理证实。治疗指征包括难治性疼痛、血尿、怀疑恶性肿瘤、肿瘤体积大、自发性破裂以及影像学表现不明确的肿瘤,这些肿瘤需要进行鉴别诊断以排除恶性肿瘤。回顾分析了患者的年龄、性别、肿瘤大小、多发性、影像学特征、与 TSC 的关系以及治疗方式等参数。
患者的年龄为 50.6 岁,平均肿瘤大小为 3.5cm。首发症状为腰痛、血尿、自发性破裂和疲劳。97 例(75.2%)为偶然发现。100 例(77.5%)为女性。68 例行保留肾单位手术(NSS),35 例行根治性肾切除术,26 例行血管栓塞术。12 例(9.3%)患者伴有 TSC。在平均 64.8 个月的随访期间,无患者发生肾功能损害。伴有 TSC 的患者发病年龄更小,肿瘤更大、双侧、多发。
肿瘤特征、与 TSC 的关系以及治疗方式的不同,导致了患者临床表现和治疗结果的显著差异。鉴于 AML 的良性性质,在决定主动监测或预防性干预时,应考虑这些参数。