title: Misstaging in nephroblastoma. Causes and consequences. A report of the Sixth Nephroblastoma Trial and Study of the International Society of Paediatric Oncology.
abstract: In the Wilms tumour trials and studies of the International Society of Paediatric Oncology (SIOP), the postoperative treatment is based on the extension (stage) and the histological type. Incorrect staging results in under- or overtreatment. The authors studied the causes and consequences of misstaging in SIOP 6. In this study, the final stage was defined by a central panel of pathologists after review of the surgical and histopathological forms and study of representative microscopical sections. In 46 out of 509 trial patients there was a discrepancy between the final stage and the stage determined at the participating centres: 33 patients were understaged of whom 27 survived more than 5 years (18% died) and 13 patients were overstaged of whom 11 survived more than 5 years (15.3% died). All children with tumour extension into the renal pelvis and treated as stage I instead of stage II survived without evidence of disease. Therefore, it was decided to treat these children in the next study as a stage I. In 17 cases the treatment was based on the surgical stage before the pathological stage was known or the treatment was given according to the stage determined by the local pathologist without waiting for the panel review. The numbers are too small to conclude on the consequences of overtreatment on late effects. For all clinicians the main and most important conclusion of this study is: wait for the final pathology report before initiating postoperative therapy.
authors.affiliation: Department of Paediatric Oncology and Pathology, Academic Medical Center, University of Amsterdam, Emma Kinderziekenhuis AMC, Amsterdam, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: de Kraker
authors.forename: J F
authors.initials: JF
authors.lastname: Delemarre
authors.forename: M R
authors.initials: MR
authors.lastname: Lilien
authors.forename: M F
authors.initials: MF
authors.lastname: Tournade
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D008297:Male; D009367:Neoplasm Staging; D009392:Nephrectomy; D015203:Reproducibility of Results; D015996:Survival Rate; D009396:Wilms Tumor / Q000401:mortality / Q000473:pathology* / Q000601:surgery