title: Persistent complete response after single-agent sunitinib treatment in a case of TFE translocation positive relapsed metastatic pediatric renal cell carcinoma.
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.
authors.affiliation: Department of Oncology, Great Ormond Street Hospital, UCL Institute of Child Health, London, UK.
authors.forename: Tanzina
authors.initials: T
authors.lastname: Chowdhury
authors.forename: Kathryn
authors.initials: K
authors.lastname: Prichard-Jones
authors.forename: Neil J
authors.initials: NJ
authors.lastname: Sebire
authors.forename: Nelly
authors.initials: N
authors.lastname: Bier
authors.forename: Abraham
authors.initials: A
authors.lastname: Cherian
authors.forename: Maureen O
authors.initials: MO
authors.lastname: Sullivan
authors.forename: Anne
authors.initials: A
authors.lastname: O'Meara
authors.forename: John
authors.initials: J
authors.lastname: Anderson
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics*; D002292:Carcinoma, Renal Cell / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D007211:Indoles / Q000627:therapeutic use*; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D008175:Lung Neoplasms / Q000188:drug therapy* / Q000235:genetics / Q000556:secondary; D008207:Lymphatic Metastasis; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D011379:Prognosis; D011758:Pyrroles / Q000627:therapeutic use*; D012074:Remission Induction; D000077210:Sunitinib; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic / Q000235:genetics*
chemical_list: D000970:Antineoplastic Agents; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D007211:Indoles; D011758:Pyrroles; C069203:TFE3 protein, human; D000077210:Sunitinib