Albersheim-Carter Jacob, Klein Molly, Murugan Paari, Weight Christopher J
University of Minnesota Medical School, Minneapolis, MN, USA.
Case Rep Urol. 2016;2016:3420741. doi: 10.1155/2016/3420741. Epub 2016 Dec 14.
Angiomyolipomas are the most common benign tumor of the kidney, associated with Tuberous Sclerosis in 20% of cases and arising sporadically in 80% of cases. Renal angiomyolipomas are neoplasms of mesenchymal origin with varying proportions of vasculature, smooth muscle spindle cells, and adipocytes, making management of such neoplasms a challenging endeavor. Possible management options include partial or radical nephrectomy and segmental renal artery embolization. A 61-year-old woman admitted for a large retroperitoneal hemorrhage was discovered to have a giant, sporadic, 3818.3 g, 30.0 × 26.5 × 18.0 cm left perinephric angiomyolipoma. Given her hemodynamic instability upon presentation, she underwent segmental arterial embolization, followed by an open left partial nephrectomy. Ten-month follow-up revealed no noticeable loss of renal function. Literature review revealed occasional renal angiomyolipomas of comparable size, with all angiomyolipomas larger than this requiring treatment with radical nephrectomy. We show that nephron-sparing surgery may be considered in the treatment of even the largest of renal angiomyolipomas.
血管平滑肌脂肪瘤是最常见的肾脏良性肿瘤,20%的病例与结节性硬化症相关,80%的病例为散发性。肾血管平滑肌脂肪瘤是间叶组织来源的肿瘤,由不同比例的血管、平滑肌梭形细胞和脂肪细胞组成,因此对这类肿瘤的治疗具有挑战性。可能的治疗选择包括部分或根治性肾切除术以及节段性肾动脉栓塞术。一名因大量腹膜后出血入院的61岁女性被发现患有一个巨大的、散发性的、重3818.3克、大小为30.0×26.5×18.0厘米的左肾周血管平滑肌脂肪瘤。鉴于她就诊时血流动力学不稳定,她接受了节段性动脉栓塞术,随后进行了开放性左肾部分切除术。十个月的随访显示肾功能没有明显丧失。文献回顾发现偶尔有大小相当的肾血管平滑肌脂肪瘤,所有比这个更大的血管平滑肌脂肪瘤都需要进行根治性肾切除术治疗。我们表明,即使是最大的肾血管平滑肌脂肪瘤,在治疗时也可考虑保留肾单位手术。