Cancer medicine · 2026
Clinical Outcomes, Treatment Patterns, and Molecular Heterogeneity of TFE3-Rearranged Renal Cell Carcinoma: A Multicenter Real-World Study.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Zhu Shuxuan, Zhou Siqi, Tian Xi, Wang Jun, Wang Ze, Jiang Jun, Liu Qiuli, Gou Xin, Zhou Xiang, Gu Jun, Yang Jianfeng, Chen Jianhui, Chen Shushang, Wang Wenfeng, Anwaier Aihetaimujiang, Chang Kun, Qu Yuanyuan, Brugarolas James, Xu Wenhao, Zhang Hailiang, Ye Dingwei
Аннотация
BACKGROUND: TFE3-rearranged renal cell carcinoma (TFE3-rRCC) is uncommon and encompasses biologically diverse tumors, while evidence to guide systemic treatment remains sparse. We examined clinical outcomes, treatment patterns, and molecular features in a multicenter real-world cohort.
METHODS: We reviewed 151 patients with TFE3-rRCC treated at eight centers in China. Treatment analyses included the 59 patients who had metastatic or relapsed disease and sufficiently detailed treatment records. Response was assessed by RECIST version 1.1. Kaplan-Meier methods were used for time-to-event outcomes, and potential confounding was explored with Cox regression.
RESULTS: Median first-line progression-free survival (PFS) was 6.1 months among the 59 treated patients. PFS was longer with first-line ICI/TKI therapy than with TKI monotherapy (log-rank p = 0.034). In an exploratory multivariable model, distant lymph-node metastasis was associated with shorter overall survival (HR, 5.22; 95% CI, 1.17-23.40; p = 0.031). In a small subgroup comparison, NONO-TFE3 tumors had a relatively immune-depleted transcriptomic profile and lower disease control with ICI/TKI therapy.
CONCLUSIONS: TFE3-rRCC is a heterogeneous disease with poor survival and a variable therapeutic response. ICI/TKI combinations had modest activity; newer approaches are needed.
METHODS: We reviewed 151 patients with TFE3-rRCC treated at eight centers in China. Treatment analyses included the 59 patients who had metastatic or relapsed disease and sufficiently detailed treatment records. Response was assessed by RECIST version 1.1. Kaplan-Meier methods were used for time-to-event outcomes, and potential confounding was explored with Cox regression.
RESULTS: Median first-line progression-free survival (PFS) was 6.1 months among the 59 treated patients. PFS was longer with first-line ICI/TKI therapy than with TKI monotherapy (log-rank p = 0.034). In an exploratory multivariable model, distant lymph-node metastasis was associated with shorter overall survival (HR, 5.22; 95% CI, 1.17-23.40; p = 0.031). In a small subgroup comparison, NONO-TFE3 tumors had a relatively immune-depleted transcriptomic profile and lower disease control with ICI/TKI therapy.
CONCLUSIONS: TFE3-rRCC is a heterogeneous disease with poor survival and a variable therapeutic response. ICI/TKI combinations had modest activity; newer approaches are needed.