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Vascular endothelial growth factor-targeted therapy for the treatment of adult metastatic Xp11.2 translocation renal cell carcinoma
Article first published online: 3 NOV 2010
Copyright © 2010 American Cancer Society
Volume 116, Issue 22, pages 5219–5225, 15 November 2010
How to Cite
Choueiri, T. K., Lim, Z. D., Hirsch, M. S., Tamboli, P., Jonasch, E., McDermott, D. F., Cin, P. D., Corn, P., Vaishampayan, U., Heng, D. Y.C. and Tannir, N. M. (2010), Vascular endothelial growth factor-targeted therapy for the treatment of adult metastatic Xp11.2 translocation renal cell carcinoma. Cancer, 116: 5219–5225. doi: 10.1002/cncr.25512
- Issue published online: 3 NOV 2010
- Article first published online: 3 NOV 2010
- Manuscript Accepted: 27 APR 2010
- Manuscript Revised: 6 APR 2010
- Manuscript Received: 1 MAR 2010
- vascular endothelial growth factor (VEGF);
- adult translocation renal cell carcinoma (RCC);
- targeted therapy;
- kidney cancer
Adult “translocation” renal cell carcinoma (RCC), bearing transcription factor E3 (TFE3) gene fusions at Xp11.2, is a recently recognized, unique entity for which prognosis and therapy remain poorly understood. In the current study, the authors investigated the effect of vascular endothelial growth factor (VEGF)-targeted therapy in this distinct subtype of RCC.
A retrospective review was conducted to describe the clinical characteristics and outcome of adult patients with metastatic Xp11.2 RCC who had strong TFE3 nuclear immunostaining and received anti-VEGF therapy. Tumor response to anti-VEGF therapy was evaluated using Response Evaluation Criteria in Solid Tumors (RECIST) criteria. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival (OS) distributions.
Fifteen patients were identified, of whom 10, 3, and 2 received sunitinib, sorafenib, and monoclonal anti-VEGF antibodies, respectively. The median follow-up was 19.1 months, the median age of the patients was 41 years, and the female:male ratio was 4:1. Initial histologic description included clear cell (n = 8 patients), papillary (n = 1 patient), or mixed clear cell/papillary RCC (n = 6 patients). Five patients had received prior systemic therapy. Five patients had undergone fluorescent in situ hybridization analysis and all demonstrated a translocation involving chromosome Xp11.2. When treated with VEGF-targeted therapy, 3 patients achieved a partial response, 7 patients had stable disease, and 5 patients developed progressive disease. The median PFS and OS of the entire cohort were 7.1 months and 14.3 months, respectively.
Adult-onset, translocation-associated metastatic RCC is an aggressive disease that affects a younger population of patients with a female predominance. In the current study, VEGF-targeted agents appeared to demonstrate some efficacy. Cancer 2010. © 2010 American Cancer Society.