Steiner M S, Goldman S M, Fishman E K, Marshall F F
Department of Urology, Johns Hopkins University School of Medicine, Baltimore, Maryland.
J Urol. 1993 Dec;150(6):1782-6. doi: 10.1016/s0022-5347(17)35895-0.
Of 35 patients with 48 angiomyolipomas 24 patients were followed clinically to determine the natural history of angiomyolipoma. Average patient age at presentation was 50 years (range 17 to 74) and of the patients 94% were women, 17% had tuberous sclerosis and 25% overall had bilateral disease. The patients could be divided into 2 distinct groups based on tumor size of 4 cm. or less and greater than 4 cm. Those with tumors less than 4 cm. were less likely to be symptomatic (24%) and patients with angiomyolipomas greater than 4 cm. were more often symptomatic (52%). No surgery was required for tumors less than 4 cm. but for 30% of the tumors greater than 4 cm. surgical intervention was necessary. Unlike any previously reported large series this study included radiological and historical followup available for 24 patients with angiomyolipoma with a mean followup time of 4 years (range 0.5 to 14). Moreover, to our knowledge we report for the first time documented growth during the study period of 27% of angiomyolipomas less than 4 cm. (4 of 15 tumors) and 46% of angiomyolipomas greater than 4 cm. (6 of 13 tumors). All patients with tumors less than 4 cm. were asymptomatic and only 1 required surgery. In contrast, tumors greater than 4 cm. were more frequently symptomatic (46%) and required surgery (54%). Patients with tuberous sclerosis and angiomyolipomas were distinctly different from patients with angiomyolipoma only, since they tended to present at a younger age, had a higher incidence of bilateral renal involvement, were more symptomatic, had larger tumors that were more likely to grow, and frequently required surgery. Based on this study, a modified approach to the current management of angiomyolipoma is recommended.
在35例患有48个血管平滑肌脂肪瘤的患者中,对24例患者进行了临床随访,以确定血管平滑肌脂肪瘤的自然病史。患者就诊时的平均年龄为50岁(范围17至74岁),其中94%为女性,17%患有结节性硬化症,25%总体患有双侧病变。根据肿瘤大小是否小于或等于4厘米,患者可分为两个不同的组。肿瘤小于4厘米的患者出现症状的可能性较小(24%),而肿瘤大于4厘米的血管平滑肌脂肪瘤患者出现症状的频率更高(52%)。肿瘤小于4厘米的患者无需手术,但30%肿瘤大于4厘米的患者需要手术干预。与以往任何报道的大型系列研究不同,本研究包括了24例血管平滑肌脂肪瘤患者的影像学和病史随访,平均随访时间为4年(范围0.5至14年)。此外,据我们所知,我们首次报告了在研究期间,27%小于4厘米的血管平滑肌脂肪瘤(15个肿瘤中的4个)和46%大于4厘米的血管平滑肌脂肪瘤(13个肿瘤中的6个)有记录的生长情况。所有肿瘤小于4厘米的患者均无症状,只有1例需要手术。相比之下,肿瘤大于4厘米的患者更常出现症状(46%),且需要手术(54%)。患有结节性硬化症和血管平滑肌脂肪瘤的患者与仅患有血管平滑肌脂肪瘤的患者明显不同,因为他们往往发病年龄较轻,双侧肾脏受累的发生率较高,症状更多,肿瘤更大且更有可能生长,并且经常需要手术。基于本研究,建议对当前血管平滑肌脂肪瘤的管理方法进行改进。