Renal neoplasms in adult survivors of childhood Wilms tumor

Purpose: Survivors of childhood Wilms tumor have been followed by large col laborative studies for approximately 31 years. In this time a number of sec ond malignant neoplasms have been documented in these Wilms tumor survivors and they are at higher risk for such development compared with the general population. To our knowledge no renal neoplasms have been previously repor ted in patients successfully treated for Wilms tumor in childhood. Materials and Methods: We reviewed the cases of 4 adults in whom Wilms tumo r was treated in childhood by radical nephrectomy and adjuvant therapy and who presented to our institution with complex cystic or solid renal masses in the contralateral kidney. Parameters, including patient age at Wilms tum or diagnosis, Wilms tumor treatment modalities, age at second malignant neo plasm presentation and resected renal lesion pathology were outlined. A tho rough literature review was performed to identify the development of renal malignancies as second malignant neoplasms in survivors of Wilms tumor in c hildhood. Results: The International Society of Pediatric Oncology and National Wilms Tumor Study have followed patients treated for Wilms tumor for no greater than 31 years. Renal neoplasms, including 2 renal cell carcinomas, 1 oncocy toma and 1 atypical cyst, in the solitary remaining kidney of relatively yo ung adults 34 to 50 years old who were treated for Wilms tumor greater than 31 years ago were successfully treated with partial nephrectomy at our ins titution. Neither the International Society of Pediatric Oncology nor Natio nal Wilms Tumor Study has identified renal cell carcinoma as a second malig nant neoplasm in patients successfully treated for Wilms tumor. Conclusions: Our experience suggests that relatively young adults with a hi story of childhood treatment for Wilms tumor may be at increased risk for r enal neoplasms at ages not yet achieved by those enrolled in large multicen ter trials. This possibility should be considered when planning the long-te rm followup of these patients. The potential to develop this type of second malignant neoplasm again raises the clinical issue of performing a primary nephron sparing procedure in children presenting with Wilms tumor.