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.
Written by:
Chowdhury T, Prichard-Jones K, Sebire NJ, Bier N, Cherian A, Sullivan MO, O'Meara A, Anderson J. Are you the author?
Departments of Oncology, Histopathology, 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.
Reference: J Pediatr Hematol Oncol. 2013 Jan;35(1):e1-3.
doi: 10.1097/MPH.0b013e318266bf34
PubMed Abstract
PMID: 22995920
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