Forty percent of renal cell carcinomas (RCCs) in childhood are characterized by translocation involving transcription factor E3 (TFE3) family members. Here, we describe a case of TFE3-positive RCC in which metastatic relapse to the mediastinal lymph nodes and pulmonary nodules was treated with single-agent sunitinib, a multitargeted tyrosine inhibitor. Complete radiologic remission was achieved after only 3 courses of treatment, and surgical exploration of metastases failed to identify any residual viable disease. The published experience of sunitinib in TFE-RCC is limited, and prospective evaluation of its activity in a larger number of patients is warranted.
Departments of *Oncology
§Urology, Great Ormond Street Hospital
†Unit of Molecular Haematology and Cancer Biology, UCL Institute of Child Health, London, UK
∥Our Lady’s Children’s Hospital, Dublin, Republic of Ireland
The authors declare no conflict of interest.
Reprints: John Anderson, MD, PhD, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK (e-mail: firstname.lastname@example.org)
Received September 13, 2011
Accepted June 26, 2012