title: The relationship between the site of metastases and outcome in children with stage IV Wilms Tumor: data from 3 European Pediatric Cancer Institutions.
abstract: The aim of this study was to analyze in detail the site of metastasis of stage 4 Wilms tumor (WT) and its correlation with outcome. The databases from 3 major European pediatric cancer institutions were screened for children with WT between 1994 and 2011. Of 208 children identified, 31 (14.9%) had metastases at diagnosis. The lung was affected in 29 children (93.5%) and the liver in 6 children (19.4%). Twenty-seven children (87.1%) had metastases isolated to 1 organ, with the lung being the most common site (80.7%). Five-year overall survival was significantly better in those children with distant disease in either lung or liver (95.8%) compared with those affected in both lung and liver (57.1%, P=0.028). Further, prognostic markers were the response of metastases to preoperative chemotherapy (P=0.0138), high-risk histology (P=0.024), and local stage (P=0.026). Five-year overall survival was 82.1% and 5-year event-free survival was 67.9%. The overall follow-up time was 74.1 and 87.2 (2 to 151) months among survivors, and the treatment-related complication rate was 16.7%. In conclusion, in our series of stage 4 WT, prognosis was excellent if histology was favorable, metastatic disease was isolated to either lungs or liver, and if metastases responded to preoperative chemotherapy.
authors.affiliation: *Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University Munich §Children's Hospital ‡Center of Pediatric Surgery, Hannover Medical School, Hannover ∥Department of Pediatric Oncology, University of Saarland, Homburg/Saar, Germany †Virgen del Rocío Children's Hospital, Seville, Spain.
authors.forename: Michael
authors.initials: M
authors.lastname: Berger
authors.forename: Israel
authors.initials: I
authors.lastname: Fernandez-Pineda
authors.forename: Rosa
authors.initials: R
authors.lastname: Cabello
authors.forename: Gema Lucía
authors.initials: GL
authors.lastname: Ramírez-Villar
authors.forename: Catalina
authors.initials: C
authors.lastname: Márquez-Vega
authors.forename: Rainer
authors.initials: R
authors.lastname: Nustede
authors.forename: Christin
authors.initials: C
authors.lastname: Linderkamp
authors.forename: Irene
authors.initials: I
authors.lastname: Schmid
authors.forename: Olaf
authors.initials: O
authors.lastname: Neth
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: de Agustin
authors.forename: Dietrich
authors.initials: D
authors.lastname: von Schweinitz
authors.forename: Martin
authors.initials: M
authors.lastname: Lacher
authors.forename: Jochen
authors.initials: J
authors.lastname: Hubertus
mesh_terms: D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D013030:Spain; D016896:Treatment Outcome; D009396:Wilms Tumor / Q000401:mortality* / Q000473:pathology* / Q000628:therapy