title: Management of adults with Wilms' tumor: recommendations based on international consensus.
abstract: Since Wilms' tumor occurs rarely in adults, there are no standard treatments available. Most adult patients will be diagnosed unexpectedly following nephrectomy for presumed renal cell carcinoma. Outcome for adults is inferior compared with children, although better results are reported when treated within pediatric trials. Multiple factors, including the unfamiliarity of adult oncologists and pathologists with Wilms' tumors, lack of standardized treatment and consequent delays in initiating the appropriate risk-adapted therapy, may contribute to the poor outcome. A standardized approach for the management of adult Wilms' tumors is proposed with the aim to limit treatment delay after surgery and encourage a uniform approach for this rare disease and thereby improve survival. These recommendations are based on discussions held with representatives of the renal tumor committees of the Society of Paediatric Oncology and Children's Oncology Group, and have been updated with a review of more recently published institutional and trial experience of adults treated on pediatric protocols. They provide a critical evaluation of the current evidence for the management of adult Wilms' tumors and propose details of how current pediatric therapeutic guidelines could be adapted for use in adults.
authors.affiliation: Deptartment of Oncology/Hematology, Erasmus MC-Sophia Children's Hospital, Dr Molewaterplein, 603015 GJ, Rotterdam, The Netherlands.
authors.forename: Heidi
authors.initials: H
authors.lastname: Segers
authors.forename: Marry M
authors.initials: MM
authors.lastname: van den Heuvel-Eibrink
authors.forename: Kathy
authors.initials: K
authors.lastname: Pritchard-Jones
authors.forename: Max J
authors.initials: MJ
authors.lastname: Coppes
authors.forename: Michael
authors.initials: M
authors.lastname: Aitchison
authors.forename: Christophe
authors.initials: C
authors.lastname: Bergeron
authors.forename: Beatriz
authors.initials: B
authors.lastname: de Camargo
authors.forename: Jeffrey S
authors.initials: JS
authors.lastname: Dome
authors.forename: Paul
authors.initials: P
authors.lastname: Grundy
authors.forename: Gemma
authors.initials: G
authors.lastname: Gatta
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
authors.forename: Paul
authors.initials: P
authors.lastname: Grundy
authors.forename: John A
authors.initials: JA
authors.lastname: Kalapurakal
authors.forename: Jan
authors.initials: J
authors.lastname: de Kraker
authors.forename: Elizabeth J
authors.initials: EJ
authors.lastname: Perlman
authors.forename: Harald
authors.initials: H
authors.lastname: Reinhard
authors.forename: Filippo
authors.initials: F
authors.lastname: Spreafico
authors.forename: Gordan
authors.initials: G
authors.lastname: Vujanic
authors.forename: Anne B
authors.initials: AB
authors.lastname: Warwick
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D011379:Prognosis; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology / Q000601:surgery*; D055815:Young Adult