title: Diagnosis and treatment of renal cell carcinoma in children: a report from the Polish pediatric rare tumor study group.
abstract: BACKGROUND
  Renal cell carcinoma (RCC) in children is rare, accounting for approximately 1.9-6% of all pediatric renal malignancies. The aim of this study was to transmit our experience in the treatment of RCC in Polish children.
  
  
  METHODS
  Clinical data from 21 children (6.3-18 years old) with RCC treated between 1992 and 2009 at Polish pediatric oncological centers were analyzed.
  
  
  RESULTS
  In 2 patients, RCC developed as a second malignancy after neuroblastoma or astrocytoma fibrillare, respectively. In 6 patients, initial diagnoses based on imaging studies were unilateral Wilms' tumor, leading to preoperative chemotherapy. The remaining patients underwent surgery at the beginning of treatment. According to the AJCC/TNM staging system, 14 patients had stage I, 5-II, 1-III, and 1-IV. Nephrectomy was performed in 19 patients, heminephrectomy in one, and biopsy in another. Histopathological diagnoses were clear-cell RCC (18 patients), papillary RCC (2 patients), and chromophobe RCC (1 patient). 10 patients were treated with chemotherapy, with or without IL-2, INFα, and antiangiogenic agents. 2 patients died due to disease progression.
  
  
  CONCLUSIONS
  RCC in children is mostly operable at diagnosis, resulting in good prognosis. The role of adjuvant chemo- and immunotherapies is unclear. Neoadjuvant chemotherapy proven for children with Wilms' tumors is ineffective, but the delay in adequate therapy did not worsen the outcome if complete nephrectomy is done subsequently.
authors.affiliation: Department of Pediatrics, Hematology, Oncology and Endocrinology, Medical University of Gdansk, Poland. tsten@gumed.edu.pl
authors.forename: T
authors.initials: T
authors.lastname: Stachowicz-Stencel
authors.forename: E
authors.initials: E
authors.lastname: Bien
authors.forename: A
authors.initials: A
authors.lastname: Balcerska
authors.forename: J
authors.initials: J
authors.lastname: Godzinski
authors.forename: A
authors.initials: A
authors.lastname: Synakiewicz
authors.forename: M
authors.initials: M
authors.lastname: Perek-Polnik
authors.forename: A
authors.initials: A
authors.lastname: Kurylak
authors.forename: W
authors.initials: W
authors.lastname: Pietras
authors.forename: M
authors.initials: M
authors.lastname: Kuzmicz
authors.forename: A
authors.initials: A
authors.lastname: Mizia-Malarz
authors.forename: A
authors.initials: A
authors.lastname: Rybczynska
authors.forename: J
authors.initials: J
authors.lastname: Nurzynska-Flak
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D002292:Carcinoma, Renal Cell / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D017024:Chemotherapy, Adjuvant; D002648:Child; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery*; D008297:Male; D020360:Neoadjuvant Therapy; D009361:Neoplasm Invasiveness; D009367:Neoplasm Staging; D016609:Neoplasms, Second Primary / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D009392:Nephrectomy; D012189:Retrospective Studies