title: Metastatic renal cell carcinoma in children and adolescents:  a 30-year unsuccessful story.
abstract: 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.
  
  
  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.
authors.affiliation: Department of Pediatric, Second University of Napoli, Napoli, Italy. mecneide.mendes@cehope.com.br
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Fiorina
authors.initials: F
authors.lastname: Casale
authors.forename: Monica
authors.initials: M
authors.lastname: Terenziani
authors.forename: Amalia
authors.initials: A
authors.lastname: Schiavetti
authors.forename: Paolo
authors.initials: P
authors.lastname: Pierani
authors.forename: Luigi
authors.initials: L
authors.lastname: Piva
authors.forename: Daniela
authors.initials: D
authors.lastname: Cuzzubbo
authors.forename: Maria D
authors.initials: MD
authors.lastname: De Pasquale
authors.forename: Elvira
authors.initials: E
authors.lastname: Pota
authors.forename: Alessandro
authors.initials: A
authors.lastname: Inserra
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
mesh_terms: D000293:Adolescent; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000235:genetics; D002292:Carcinoma, Renal Cell / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D002648:Child; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D009362:Neoplasm Metastasis; D012189:Retrospective Studies
chemical_list: D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; C069203:TFE3 protein, human