Adult Wilms' tumor: A monoinstitutional experience and a review of the literature

BACKGROUND: The authors reviewed their institutional experience regarding adult patients with Wilms' tumor (WT) to assess their clinical characteristics and compliance with respect to children's treatment guidelines. METHODS: A total of 17 adult patients (median age at the time of diagnosis of 17.5 years; range, 16-29 years) were referred to the study institute between 1983 and 2001 and were followed for a median of 131 months. The treatment modality was planned according to the two consecutive Italian protocols for WT that were active during the referral years. The patients were staged according to the National Wilms Tumor Study-4 (NWTS) staging system as follows: eight patients had Stage II disease, four patients had Stage III disease, and five patients had Stage IV disease. RESULTS: All the patients but one underwent nephrectomy, with three incomplete surgeries performed. Two patients with Stage II disease were treated elsewhere with nephrectomy only and they were admitted to the study institution at the time of disease recurrence. Anaplasia was found to be present in only one patient with Stage IV disease. The authors noted 9 cases of disease recurrence or progression occurring during treatment and 6 of these 9 patients died of their disease, with an overall survival rate of 62.4% at 5 years. CONCLUSIONS: Compared with children, adults with WT are reported to have a worse prognosis. In the current study, the authors found that poor compliance with specific therapeutic guidelines may contribute to this poorer outcome. Because of the rarity of this disease, adults with WT are at a risk of either undertreatment or incorrect treatment.