A case of adult Wilms' tumor --review of the Japanese literature

The patient was a 23-year-old-woman who was referred to our hospital with chief complaints of right flank pain and macroscopic hematuria. Right radical nephrectomy was performed with the diagnosis of right renal cancer. Histopathological examination revealed Wilms' tumor of favorable histology, stage I according to the National Wilms Tumor Study classification. She received adjuvant chemotherapy consisting of actinomycin D and vincristine at another hospital. One year and three months later, she developed lung metastasis. She underwent partial pulmonary resection, and then received chemotherapy. She is presently disease-free more than forty months after the initial operation. Wilms' tumor is rare in adults and has a poor prognosis compared with that in children. We analyzed 112 cases in the Japanese literature from 1981 to 2004. The mean age was 36.9 years, with males and females equally affected. No difference was found between the left and right sides in frequency of tumor. We also examined the relationship between histological features and prognosis based on 43 Japanese reports. Twenty-two of the 43 (51%) cases had unfavorable histology. The 2-year survival rate with unfavorable histology was 18%, while that with favorable histology was 87%. The 2-year survival rates for stages under II and over III were 67% and 27%, respectively. Based on these findings, we conclude that the prognosis of adult Wilms' tumor is very poor since many patients have unfavorable histology and no effective treatment guidelines have been established.