O J Kessler1, J F Siegel, W A Brock. 1. Department of Urology, Schneider Children's Medical Center of Israel, Petah Tiqva.
Abstract
OBJECTIVES: To better characterize intrarenal neuroblastoma. METHODS: We retrospectively reviewed the records of 5 children with neuroblastoma initially diagnosed as Wilms' tumor. RESULTS: Five patients, aged 11 months to 3.5 years, were preoperatively diagnosed as having intrarenal Wilms' tumor, prior to operative or histopathologic diagnosis of neuroblastoma. In 3 children the diagnosis of neuroblastoma involving the kidney was confirmed; in the fourth child subcutaneous metastatic disease precluded biopsy confirmation of the intrarenal tumor; and in the fifth child the kidney was severely displaced but not actually involved by neuroblastoma. All patients were hypertensive and 4 patients had elevated urinary catechol levels. Molecular genetic studies showed N-myc amplification in both patients who were evaluated. All patients had poorly differentiated histopathology. One patient is presently alive at 8 months with recurrent disease and another patient is free of disease 13 years after diagnosis. The remaining 3 patients died of the malignancy. CONCLUSIONS: Intrarenal neuroblastoma is a rare entity that clinically and radiographically may resemble Wilms' tumor. Our limited experience indicates that intrarenal neuroblastoma is an aggressive malignancy, and long-term survival is rare.
OBJECTIVES: To better characterize intrarenal neuroblastoma. METHODS: We retrospectively reviewed the records of 5 children with neuroblastoma initially diagnosed as Wilms' tumor. RESULTS: Five patients, aged 11 months to 3.5 years, were preoperatively diagnosed as having intrarenal Wilms' tumor, prior to operative or histopathologic diagnosis of neuroblastoma. In 3 children the diagnosis of neuroblastoma involving the kidney was confirmed; in the fourth child subcutaneous metastatic disease precluded biopsy confirmation of the intrarenal tumor; and in the fifth child the kidney was severely displaced but not actually involved by neuroblastoma. All patients were hypertensive and 4 patients had elevated urinary catechol levels. Molecular genetic studies showed N-myc amplification in both patients who were evaluated. All patients had poorly differentiated histopathology. One patient is presently alive at 8 months with recurrent disease and another patient is free of disease 13 years after diagnosis. The remaining 3 patients died of the malignancy. CONCLUSIONS: Intrarenal neuroblastoma is a rare entity that clinically and radiographically may resemble Wilms' tumor. Our limited experience indicates that intrarenal neuroblastoma is an aggressive malignancy, and long-term survival is rare.
Authors: Matthew J Murray; Katie L Raby; Harpreet K Saini; Shivani Bailey; Sophie V Wool; Jane M Tunnacliffe; Anton J Enright; James C Nicholson; Nicholas Coleman Journal: Cancer Epidemiol Biomarkers Prev Date: 2014-11-21 Impact factor: 4.254