Simmons J L, Hussain S A, Riley P, Wallace D M A
University Hospital Birmingham, Edgbaston, UK.
Oncol Rep. 2003 Jan-Feb;10(1):237-41.
Renal angiomyolipomas are common in patients with tuberous sclerosis complex (TSC), and the risk of severe haemorrhage from these angiomyolipomas can become substantial. This case illustrates a potentially life-threatening condition due to the development of a large aneurysm within an angiomyolipoma, which was discovered within 14 months of her screening renal ultrasound scan. Renal arterial embolisation and renal sparing surgery resulted in good recovery. Clear guidelines for the screening, surveillance, and treatment of angiomyolipomas in patients with TSC are required. This includes the appropriate frequency of surveillance for patients in different age groups and at different stages of angiomyolipoma development, based on a growing knowledge of the natural history of this condition, since growth of renal angiomyolipomas can be rapid and asymptomatic. Computed tomography or magnetic resonance imaging may be required to demonstrate complications in large lesions, as three ultrasound examinations in this patient failed to detect the large aneurysm which had developed. Angiogenesis inhibitors could potentially play a role in preventing the development of angiomyolipomas, which could improve the prognosis for patients with TSC and therefore warrants investigation through phase II/III clinical trials.
肾血管平滑肌脂肪瘤在结节性硬化症（TSC）患者中很常见，这些血管平滑肌脂肪瘤发生严重出血的风险可能很大。本病例说明了由于血管平滑肌脂肪瘤内出现一个大动脉瘤而导致的潜在危及生命的情况，该动脉瘤在她的肾脏超声筛查扫描后的14个月内被发现。肾动脉栓塞和保留肾手术导致患者恢复良好。需要针对TSC患者的血管平滑肌脂肪瘤的筛查、监测和治疗制定明确的指南。这包括根据对这种疾病自然史的不断了解，为不同年龄组和处于血管平滑肌脂肪瘤发展不同阶段的患者确定适当的监测频率，因为肾血管平滑肌脂肪瘤的生长可能迅速且无症状。对于大的病变，可能需要计算机断层扫描或磁共振成像来显示并发症，因为该患者的三次超声检查均未检测到已形成的大动脉瘤。血管生成抑制剂可能在预防血管平滑肌脂肪瘤的发生中发挥作用，这可能改善TSC患者的预后，因此值得通过II/III期临床试验进行研究。
