ArticleMolecular cancer2024
Patients with ASPSCR1-TFE3 fusion achieve better response to ICI based combination therapy among TFE3-rearranged renal cell carcinoma.
Article in Molecular cancer, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Recurrent TFE3-Rearranged Renal Cell Carcinoma Responding to Pembrolizumab Plus Lenvatinib After Postoperative Pembrolizumab: A Case Report.IJU case reports · 2026Article
- ASPSCR1::TFE3-rearranged renal cell carcinomas: clinicopathologic, molecular, and survival analysis of 30 cases.Virchows Archiv : an international journal of pathology · 2026Article
- From fusion partner to clinical practice: A treatment-oriented framework for transcription factor E3 (Cancer pathogenesis and therapy · 2026Review
- TFE3-DualNet: An Interpretable Foundation Model-Based Deep Learning Ensemble for Diagnosing TFE3-Rearranged Renal Cell Carcinoma From Whole-Slide Images in a Two-Center Cohort.Cancer medicine · 2026Article
- Review
- Remarkable Response to Immunotherapy and VEGFR Inhibitor in Xp11 Translocation Renal Cell Carcinoma in a 17-Year-Old Adolescent.Case reports in medicine · 2026Article
- SV2B Promotes the Progression of TFE3-Rearranged Renal Cell Carcinoma by Interacting with HERC2 to Impede the Degradation of NF-κB Subunits.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2026Article
- Molecular and therapeutic landscape of non-clear cell renal carcinoma.Nature reviews. Urology · 2025Review
- AI-assisted diagnosis of renal cell carcinoma: educational needs and cognitive assessment based on the WHO classification 2022.BMC medical education · 2025Article
- LGI3 promotes the progression of TFE3-rearranged renal cell carcinoma through GEMIN6/AURKB axis.Oncogene · 2025Article
- [Clinicopathological features and survival analysis ofBeijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences · 2025Article
- Targeting the PD-1/PD-L1 Signaling Pathway for Cancer Therapy: Focus on Biomarkers.International journal of molecular sciences · 2025Review
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Authors and funding
23 authors.
Funding
Abstract
backgroundTFE3-rearranged renal cell carcinoma (TFE3-rRCC) is a rare but highly heterogeneous renal cell carcinoma (RCC) entity, of which the clinical treatment landscape is largely undefined. This study aims to evaluate and compare the efficacy of different systemic treatments and further explore the molecular correlates.
methodsThirty-eight patients with metastatic TFE3-rRCC were enrolled. Main outcomes included progression-free survival (PFS), overall survival, objective response rate (ORR) and disease control rate. RNA sequencing was performed on 32 tumors.
resultsPatients receiving first-line immune checkpoint inhibitor (ICI) based combination therapy achieved longer PFS than those treated without ICI (median PFS: 11.5 vs. 5.1 months, P = 0.098). After stratification of fusion partners, the superior efficacy of first-line ICI based combination therapy was predominantly observed in ASPSCR1-TFE3 rRCC (median PFS: not reached vs. 6.5 months, P = 0.01; ORR: 67.5% vs. 10.0%, P = 0.019), but almost not in non-ASPSCR1-TFE3 rRCC. Transcriptomic data revealed enrichment of ECM and collagen-related signaling in ASPSCR1-TFE3 rRCC, which might interfere with the potential efficacy of anti-angiogenic monotherapy. Whereas angiogenesis and immune activities were exclusively enriched in ASPSCR1-TFE3 rRCC and promised the better clinical outcomes with ICI plus tyrosine kinase inhibitor combination therapy.
conclusionsThe current study represents the largest cohort comparing treatment outcomes and investigating molecular correlates of metastatic TFE3-rRCC based on fusion partner stratification. ICI based combination therapy could serve as an effective first-line treatment option for metastatic ASPSCR1-TFE3 rRCC patients. Regarding with other fusion subtypes, further investigations should be performed to explore the molecular mechanisms to propose pointed therapeutic strategy accordingly.
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