Systemic Therapy in Patients With Metastatic Xp11.2 Translocation Renal Cell Carcinoma |
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| Institution: | 1. Key laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing), Department of Genitourinary oncology, Peking University Cancer Hospital & Institute, Beijing, China;2. Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education, Beijing), Department of Melanoma and Sarcoma, Peking University Cancer Hospital & Institute, Beijing, China;1. Department of Urology, Wake Forest University School of Medicine, Winston-Salem, NC;2. Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC;3. Department of Epidemiology, Gillings School of Global Public Health, University of North Carolina, Chapel Hill, NC;4. Department of Urology, University of North Carolina, Chapel Hill, NC;5. Department of Radiation Oncology, University of Kansas, Kansas City, KS;6. Atrium Health, Charlotte, NC;7. Departments of Urology and Health Policy & Management, University of California, Los Angeles, CA;8. Division of Oncology, Department of Medicine, University of North Carolina, Chapel Hill, NC;1. Department of Urology and Pediatric Urology, University Hospital of Würzburg, Würzburg, Germany;2. Department of Urology, University Hospital of Tübingen, Tübingen, Germany;3. King Salman Hospital (MOH), Riyadh, Saudi Arabia;1. Department of Surgery– Urology, Piove di Sacco (PD) AULSS 6 Euganea, Padova, Italy;2. Department of Surgery, Oncology and Gastroenterology – Urology, Padova, Italy;3. Department of Nephrology, Dialysis and Kidney Transplant, International Renal Research Institute, San Bortolo Hospital, Vicenza, Italy;1. Department of Urology, Kyorin University School of Medicine, Tokyo, Japan;2. Department of Radiology, Kyorin University School of Medicine, Tokyo, Japan;3. Department of Radiology, Tokyo Women''s Medical University Medical Center East, Tokyo, Japan;1. University Hospital Munich, Department of Urology, Munich, Germany;2. Hospital Barmherzige Brüder, Department of Urology, Munich, Germany;1. Unit of Urology, A.Gemelli Hospital – IRCCS, Catholic University of the Sacred Heart – Rome, Italy;2. Unit of Pathology, A.Gemelli Hospital – IRCCS, Catholic University of the Sacred Heart – Rome, V, Italy |
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| Abstract: | BackgroundXp11.2 translocation renal cell carcinoma (Xp11.2 tRCC) is a unique subtype with poor prognosis, its response to systemic therapy is not fully understood, we evaluated the benefit of systemic therapy in these patients.Patients and MethodsBetween May 2006 and December 2019, patients diagnosed with Xp11.2 tRCC from Peking university cancer hospital were collected. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival (OS) distributions.ResultsMetastatic Xp11.2 tRCC was found in 45 patients. The median PFS and median OS was 7.4 months (4.5-8.8) and 17.9 months (12.4-24.4), respectively. First-line treatment mainly included sunitinib (n = 14), sorafenib (n = 15), axitinib (n = 6), and pazopanib (n = 5), and the median PFS of these regimens were 7.4 months, 5.4 months, 9.4 months, 8.9 months, respectively. Two patients who received Vascular endothelial growth factor receptor - tyrosine kinase inhibitor (VEGFR-TKI) plus immune checkpoint inhibitor (ICI) as first line therapy had a PFS of more than 16.6 months and more than 25.6 months, respectively. Twenty-four patients received subsequent therapies, which included VEGFR-TKI/ICI, VEGFR-TKI and mTOR inhibitor. The ORR and median PFS was 33% and 7.1 months, 7.7% and 4.3 months, 0% and 2.1 months for these treatments, respectively. The estimated median OS was 17.3 months (95% CI, 11.2 to not reached) in patients with TKI/ICI treatment and 11.0 months (95% CI, 6.1 to not reached) without TKI/ICI treatment in subsequent therapies (P = .04). Patients with serous cavity effusion or IMDC poor risk groups had significantly shorter median PFS and median OS.ConclusionMetastatic Xp11.2 tRCC is an aggressive disease. VEGFR-TKI agents appeared to demonstrate some efficacy, VEGFR-TKI /ICI combination might be a useful tool for the treatment of metastatic Xp11.2 tRCC. |
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