title: Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study.
abstract: OBJECTIVE
  In the Society of Pediatric Oncology (SIOP) 93-01 study, 30 patients older than 16 years were found to have Wilms' tumor. They were treated according to the pediatric protocol and were analyzed for clinical presentation, stage distribution, and prognosis.
  
  
  METHODS
  Patient age ranged from 16 to 62 years (median, 25.4 years). Tumor stages were defined according to SIOP, and treatment was risk-adapted according to SIOP 93-01/Society for Pediatric Oncology and Hematology (GPOH) protocol. The patients were evaluated with regard to response, toxicity, and prognosis. Specimens of all tumors were centrally reviewed.
  
  
  RESULTS
  Ten patients (33%) had metastatic disease at the time of diagnosis (liver, four patients; lung, three patients; liver and lung, three patients). The local stage distribution showed a predominance of higher stages (stage I, eight patients; stage IIN-, three patients; stage IIN+, four patients; stage III, 15 patients). Histologic studies revealed intermediate-risk in 23 of 30 tumors; two tumors were classified as high-risk; and three tumors were clear-cell sarcomas. Two of 30 patients showed a nephroblastoma and a renal cell carcinoma simultaneously in the same kidney. A complete remission was achieved in 24 patients; four patients relapsed after complete remission; and three of them reached a second remission with further treatment. Event-free survival was 57%, with an overall survival of 83% (median observation time, 4 years).
  
  
  CONCLUSIONS
  Adults can be cured in a high percentage by a multimodal treatment according to pediatric protocols. Toxicity is higher than in children, but acceptable in view of the high remission rate.
authors.affiliation: Department of Pediatric Oncology, University Hospital, Homburg/Saar, Germany. harald.reinhard@uniklinik-saarland.de
authors.forename: Harald
authors.initials: H
authors.lastname: Reinhard
authors.forename: Schahin
authors.initials: S
authors.lastname: Aliani
authors.forename: Christian
authors.initials: C
authors.lastname: Ruebe
authors.forename: Michael
authors.initials: M
authors.lastname: Stöckle
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000188:drug therapy* / Q000473:pathology* / Q000532:radiotherapy; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000188:drug therapy* / Q000473:pathology* / Q000532:radiotherapy