Application of nephron-sparing surgery in tuberous sclerosis complex associated renal angiomyolipoma
10.3760/cma.j.cn112139-20250722-00362
- VernacularTitle:保留肾单位手术在结节性硬化症相关性肾血管平滑肌脂肪瘤中的应用
- Author:
Jiang LIU
1
;
Yang ZHAO
1
;
Shiwei SUN
1
;
Songchen HAN
1
;
Zhan WANG
1
;
Yi LIU
1
;
Wenda WANG
1
;
Wenwen CHEN
1
;
Yushi ZHANG
1
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院泌尿外科，北京 100730
- Publication Type:Journal Article
- Keywords:
Surgical procedures, operative;
Tuberous sclerosis complex;
Renal angiomyolipoma;
Nephron-sparing surgery;
Renal function
- From:
Chinese Journal of Surgery
2025;63(12):1131-1136
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical outcomes of nephron-sparing surgery (NSS) for tuberous sclerosis complex(TSC) -associated renal angiomyolipoma (RAML). Methods:This retrospective case-series study analyzed the clinical data of 15 TSC-RAML patients who underwent NSS at the Department of Urology, Peking Union Medical College Hospital between April 2013 and July 2024. The cohort included 4 males and 11 females. The age at TSC diagnosis was (28.5±14.5) years (range: 5 to 62 years), and the age at first surgery was (33.3±10.8) years (range: 18 to 62 years). The maximum tumor diameter( M(IQR)) was 6.4(7.9)cm (range: 3.5 to 31.5 cm). Patient baseline characteristics, surgical approach, TSC-RAML staging, imaging findings, and laboratory data were collected. Relationships between variables were analyzed using Generalized Estimating Equations with post-hoc tests. Results:A total of 18 surgical procedures were performed on the 15 patients. These included 7 open surgeries, 9 laparoscopic surgeries, and 2 laparoscopic procedures converted to open surgery. The mean preoperative serum creatinine level was (70.2±14.2) μmol/L (range: 50 to 101 μmol/L), which increased to (99.2±29.8) μmol/L (range: 47 to 171 μmol/L) on postoperative day 1. However, at one year postoperatively, serum creatinine was (76.8±13.5)μmol/L (range: 55 to 106 μmol/L),showed no significant difference from preoperative levels ( P>0.05). At the 6-month postoperative follow-up, the Utrecht Interventional Classification stage for all treated tumors had decreased to grade 1 or 2. At the 12-month follow-up (available for 13 patients), 11 patients showed no disease progression. Conclusions:NSS is a viable treatment option for rigorously selected patients with high-stage TSC-RAML. Although NSS causes transient renal function impairment, it provides effective tumor burden control. In stringently selected patients with high-stage disease and under long-term follow-up, NSS is associated with limited long-term renal impairment.
