Katada Yoshiaki, Umehara Isao, Ohki Takemasa, Kishino Mitsuhiro, Shibuya Hitoshi
Department of Radiology, Saitama Red Cross Hospital, 8-3-33, Kamiochiai, Chuo-ku, Saitama-shi, Saitama 338-8553, Japan.
Cases J. 2009 Jul 31;2:6351. doi: 10.4076/1757-1626-2-6351.
Renal AML complicating tuberous sclerosis shows a rapid growth and its rupture is frequently associated with hemorrhagic shock as a result of profuse retroperitoneal bleeding, necessitating an aggressive therapeutic approach. This report describes the long-term clinical progress of 28 year-old woman with tuberous sclerosis with a ruptured giant AML that underwent unilateral nephrectomy, who has been followed up after treatment with concomitant application of computed tomography during selective arteriography to conserve the remaining normal renal parenchyma, and in whom the need for initiation of dialysis has been successfully avoided, with conspicuous reduction of the tumor size.
肾血管平滑肌脂肪瘤合并结节性硬化症时生长迅速,其破裂常因腹膜后大量出血而导致失血性休克,因此需要积极的治疗方法。本报告描述了一名28岁结节性硬化症女性患者的长期临床病程,该患者患有巨大的破裂性肾血管平滑肌脂肪瘤,接受了单侧肾切除术,术后在选择性动脉造影期间同时应用计算机断层扫描以保留剩余的正常肾实质,并成功避免了开始透析的需要,肿瘤大小显著减小。