title: Pediatric non-Wilms' renal tumors: a third world experience.
abstract: BACKGROUND
  Pediatric non-Wilms' renal tumors (NWRT) are poorly understood owing to their heterogeneity and relative rarity. This study aimed at auditing the outcome of the management of NWRT in a tertiary hospital in the Third World.
  
  
  METHODS
  Records of all patients (n = 68) treated for NWRT over a 32-year period (1978-2010) were reviewed retrospectively.
  
  
  RESULTS
  The major histological groups included clear cell sarcoma of the kidney (CCSK) (33.8%), mesoblastic nephroma (17.6%), cystic partially differentiated nephroblastoma (CPDN) (17.6%), intrarenal neuroblastoma (8.8%), malignant rhabdoid tumor (MRT) (7.4%), and renal cell carcinoma (RCC) (5.9%). Sixteen (69.7%) patients with CCSK and 11 (91.7%) with CPDN were aged 1-4 years. Ten (83.3%) patients with mesoblastic nephroma were aged <1 year and three (60.0%) with RCC were aged 10-14 years. Ten (43.5%) patients with CCSK and four (80.0%) with RCC had metastases at diagnosis. The sensitivity of a pretreatment Tru-Cut biopsy was 100% for MRT. All the patients with CCSK, mesoblastic nephroma, CPDN, and RCC had radical nephrectomy. Only eight (34.8%) patients with CCSK received radiotherapy. The overall 1-10-year survival rates were 52.2%, 91.7%, 75.0%, 40.0% and 0.0% for CCSK, mesoblastic nephroma, CPDN, RCC, and MRT, respectively. The overall 1-10-year survival for the entire cohort was 51.5%.
  
  
  CONCLUSIONS
  The demography and clinical presentation of pediatric NWRT, which comprises 13.6% of pediatric renal tumors in the Third World, were similar to those in the Developed World. The overall 1-10-year survival for pediatric NWRT was low.
authors.affiliation: Department of Paediatric Surgery, Nelson R. Mandela School of Medicine, University of KwaZulu-Natal, Private Bag 7, Congella 4013, South Africa. saulapw@yahoo.com
authors.forename: Peter W
authors.initials: PW
authors.lastname: Saula
authors.forename: G P
authors.initials: GP
authors.lastname: Hadley
mesh_terms: D000293:Adolescent; D002292:Carcinoma, Renal Cell / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000401:mortality / Q000601:surgery* / Q000628:therapy; D020360:Neoadjuvant Therapy; D018201:Nephroma, Mesoblastic / Q000601:surgery; D012189:Retrospective Studies; D018227:Sarcoma, Clear Cell / Q000601:surgery*; D009396:Wilms Tumor / Q000601:surgery