Metastatic renal cell carcinoma in children and adolescents: A 30-year unsuccessful story

Paolo Indolfi, Filippo Spreafico, Paola Collini, Giovanni Cecchetto, Fiorina Casale, Monica Terenziani, Amalia Schiavetti, Paolo Pierani, Luigi Piva, Daniela Cuzzubbo, Maria D. De Pasquale, Elvira Pota, Alessandro Inserra, Gianni Bisogno

Research output: Contribution to journalArticlepeer-review


BACKGROUND: Because of the rare occurrence of renal cell carcinoma (RCC) among children very little is known about this malignancy in pediatric age. We aimed adding knowledge on the clinical characteristics and outcome of metastatic (m) RCC in children and adolescents. PATIENTS AND METHODS: The series included 14 stage 4 RCC patients with a median age at diagnosis of 155.5 months, observed at the Italian Pediatric Hematology and Oncology Association (AIEOP) centers from January 1973 to November 2010. We were able to reevaluate histopatology of 11 out of the 14 patients and perform immunostaining for TFE3 in 9 out of the 11 patients. RESULTS: Of the 14 patients under study, 5 (3 girls) had a translocation morphology TFE+ RCC, 2 were reassigned as papillary type 1 or 2, respectively, 2 tumor specimens with primary clear cell histology had confirmed the initial histologic diagnosis, and 2-whose biopsy specimen was insufficient-had the diagnosis of RCC not further specified with subtyping. In the remaining 3 cases, the initial diagnosis of clear cell carcinoma was left. Overall, 6 patients received chemotherapy, 9 immunotherapy, and 2 adjuvant antiangiogenic therapy. Overall, 11 patients (78.5%) never achieved complete remission and died from progressive disease 1 to 16 months after diagnosis (median overall survival 5.5 mo). Three patients, 2 of whom received adjuvant antiangiogenic therapy, relapsed to lung at 3, 6, and 8 months after diagnosis, and died 18, 32, and 33 months after diagnosis, respectively. CONCLUSIONS: Despite their possibly different biology, childhood and adult mRCC seems to be sharing comparable outcomes. Because of the very low incidence of mRCC (about 20%) in children and adolescents, an international pediatric cooperation to address biological studies and assess the novel targeted approaches is needed.

Original languageEnglish
JournalJournal of Pediatric Hematology/Oncology
Issue number7
Publication statusPublished - Oct 2012


  • metastatic renal cell carcinoma
  • pediatric
  • rare cancer
  • targeted therapy

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Hematology


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