Prognostic variables in adult Wilms tumour.

OBJECTIVE To identify outcomes and prognostic variables that predict survival outcomes in adult Wilms tumour patients. METHODS We collected data on 128 patients with adult Wilms tumour treated between 1973 and 2006. Six cases from our 2 Canadian centres have not been previously reported. We collected data on the remaining 122 patients from published case reports or case series. Analyzed factors included age, sex, favourable or unfavourable histopathology, clinical stage (I, II, III or IV) and chemotherapy and radiotherapy received. The outcomes studied included overall survival (OS) and disease-specific survival (DSS). Univariate analysis with Kaplan-Meier actuarial methodology and multivariate analyses with Cox regression were used to determine outcomes and predictive clinical factors. RESULTS The patients' mean age was 26 (range 15-73) years. After a mean follow-up of 54 (range 2-240) months, the OS and DSS of the entire cohort were both 68%. Favourable histopathology predicted superior OS and DSS (both p < 0.001). Higher clinical stage predicted inferior OS and DSS (both p < 0.001). CONCLUSION Adult Wilms tumour has a poorer prognosis than pediatric Wilms tumour. In adults with Wilms tumour, more aggressive patient-and tumour-specific surveillance and adjunctive therapies than those advocated by pediatric National Wilms Tumor Study guidelines may be warranted, especially in patients with an unfavourable histopathology and higher clinical stage.