[Application of nephron-sparing surgery in tuberous sclerosis complex associated renal angiomyolipoma].

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.