病例報告：腎血管平滑肌脂肪瘤破裂之緊急處置
張琮昕1、陳建志1、林文州1、張奐光1、楊志東1、許炯明1、
蔡維恭1、江百凱1、周永強1、林文榮1、邱文祥1,2
1台北馬偕紀念醫院 泌尿科；2國立陽明大學 醫學院
Emergent management of renal angiomyolipoma rupture
Tsung-Hsin Chang, MD1, Chien-Chih Chen, MD, PhD1, Wen-Chou Lin, MD1, Huang-Kuang Chang, MD1, Stone Yang, MD1, Jong-Ming Hsu, MD1, Wei-Kung Tsai, MD1, Pai-Kai Chiang, MD1, Yung-Chiong Chow, MD, PhD1, Wun-Rong Lin, MD1, Wen-Hsiang Chiu, MD, PHD1,2,
1Department of Urology, Mackay Memorial Hospital, Taipei, Taiwan
2 School of Medicine, National Yang-Ming University, Taipei, Taiwan, ROC.
Abstract
Purpose: To evaluate the safety and long-term outcomes for patients with ruptured renal angiomyolipoma (AML) treated by urgent transcatheter arterial embolization (TAE) and/or emergent nephrectomy. We described the clinical presentation, treatment option and follow up course, thus to improve our experience and management to this rare but potentially lethal condition of this benign disease.
Material and Methods: We retrospectively reviewed medical charts between January 2000 and December 2016. A total of 16 patients presented with renal AMLs rupture were seen. The history, clinical presentation, treatment and prognosis of the 16 patients were analyzed. All cases underwent treatment option with either emergent TAE or nephrectomy.
Results: 16 cases presented with ruptured AMLs during the studied time. Patients were 23 to 77 years old with an average age at 47. All of the patients had spontaneous rupture except 1 patient had trauma history. 11 patients were female and 5 were male. The average size of the AML was 9 cm. Of the 16 patients, 9 received emergent TAE, 2 patients received further nephrectomy despite TAE. 4 Patients received emergent nephrectomy due to unstable hemodynamic status. The other 2 patients were treated with complete bed rest and conservative treatment. 15 patients received a mean follow up time of 30 months, no long term renal impairment, re-rupture events or mortality were reported in our study.
Discussion: Renal AMLs is a relatively benign lesion and could be treated conservatively with annual surveillance or image follow up. It should be taken into account when the size of the tumor is larger than 4 cm or when it becomes symptomatic. Spontaneous rupture leading to massive retroperitoneal hemorrhage and serious lethal condition should be treated emergently whether with TAE or nephrectomy.
In our experience, TAE is a less invasive, kidney preserving, causes minimal loss of renal function and could effectively prevent re-rupture in the long term. Initial procedure with TAE for ruptured AMLs is safe, efficient and could help to preserve the involved kidney. Only in patients with unstable hemodynamic or uncontrollable hemorrhage need to undergo nephrectomy.