Renal neoplasms in adult survivors of childhood Wilms tumor.

Abstract

Survivors of childhood Wilms tumor have been followed by large collaborative studies for approximately 31 years. In this time a number of second 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 reported in patients successfully treated for Wilms tumor in childhood. We reviewed the cases of 4 adults in whom Wilms tumor 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 tumor diagnosis, Wilms tumor treatment modalities, age at second malignant neoplasm presentation and resected renal lesion pathology were outlined. A thorough literature review was performed to identify the development of renal malignancies as second malignant neoplasms in survivors of Wilms tumor in childhood. 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 oncocytoma and 1 atypical cyst, in the solitary remaining kidney of relatively young 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 institution. Neither the International Society of Pediatric Oncology nor National Wilms Tumor Study has identified renal cell carcinoma as a second malignant neoplasm in patients successfully treated for Wilms tumor. Our experience suggests that relatively young adults with a history of childhood treatment for Wilms tumor may be at increased risk for renal neoplasms at ages not yet achieved by those enrolled in large multicenter trials. This possibility should be considered when planning the long-term 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.