INTRARENAL NEUROBLASTOMA MASQUERADING AS WILMS-TUMOR

Objectives. To better characterize intrarenal neuroblastoma. Methods. We retrospectively reviewed the records of 5 children with neuroblasto ma initially diagnosed as Wilms' tumor. Results. Five patients, aged 1 1 months to 3.5 years, were preoperatively diagnosed as having intrare nal Wilms' tumor, prior to operative or histopathologic diagnosis of n euroblastoma. In 3 children the diagnosis of neuroblastoma involving t he kidney was confirmed; in the fourth child subcutaneous metastatic d isease precluded biopsy confirmation of the intrarenal tumor; and in t he fifth child the kidney was severely displaced but not actually invo lved by neuroblastoma. All patients were hypertensive and 4 patients h ad 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 ali ve at 8 months with recurrent disease and another patient is free of d isease 13 years after diagnosis. The remaining 3 patients died of the malignancy. Conclusions. Intrarenal neuroblastoma is a rare entity tha t clinically and radiographically may resemble Wilms' tumor. Our limit ed experience indicates that intrarenal neuroblastoma is an aggressive malignancy, and long-term survival is rare. (C) 1998, Elsevier Scienc e Inc. All rights reserved.