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Persistent Complete Response After Single-agent Sunitinib Treatment in a Case of TFE Translocation Positive Relapsed Metastatic Pediatric Renal Cell Carcinoma

Chowdhury, Tanzina MD, PhD; Prichard-Jones, Kathryn MD, PhD; Sebire, Neil J. MD; Bier, Nelly BSc; Cherian, Abraham MD; Sullivan, Maureen O. MD; O’Meara, Anne MD; Anderson, John MD, PhD

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e318266bf34
Online Articles: Clinical and Laboratory Observations
Abstract

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.

Author Information

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

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: j.anderson@ich.ucl.ac.uk)

Received September 13, 2011

Accepted June 26, 2012

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