Wilms' tumor in the adult patient. Diagnosis, management, and review of the world medical literature

Adult Wilms' tumor, unlike that of childhood, is a rare disease. A total of 167 cases have been reported in the world medical literature. However, there is controversy as to whether all of these are indeed truly nephroblastomas or in actuality represent sarcomatoid renal carcinomas. The pathologic hallmarks of this neoplasms are distinctive and it is histologically similar to the childhood tumor. The therapeutic guidelines and surgical principles that govern childhood Wilms' tumor should be applied to adult Wilms' tumor. Agressive and multimodal therapy should be the hallmark of management of this rare neoplasm. Three well documented cases of Wilms' tumor in the adult patient are presented. Of these three patients, two who presented with Stage IV disease and were treated with multimodal therapy were rendered free of disease, one living at 24 months and the other dead at five months.