Adult Wilms' tumor: clinical characteristics of four cases and review of the literature

Abstract

Adult Wilms' tumor (AWT) is a rare disease. We investigated the clinical characteristics and the outcome of four patients with AWT treated by nephrectomy followed by radiation therapy and/or chemotherapy (protocol of the Fourth National Wilms' Tumor Study). Serum levels of lactic dehydrogenase (LDH), immunosuppressive acidic protein (IAP), and C-reactive protein (CRP) were above normal in all four patients. Two of three patients with unfavorable histology (UFH) had stage III or IV disease and died within 14 months of diagnosis while undergoing the chemotherapy. The other patient with UFH had a stage II tumor, which was treated with tumor-bed irradiation, and has survived for 26 months after nephrectomy. One patient with a stage I tumor and favorable histology also received chemotherapy, but she subsequently developed multiple metastases, and died 70 months after nephrectomy. Radiotherapy for metastases had palliative effects on osseous pain and tumor growth. The clinical results of the chemotherapy for AWT were poor. Analysis of more cases is needed to indicate whether or not the conventional chemotherapeutic regimen for Wilms' tumor in children requires modification to improve the outcome in AWT.