Metachronous bilateral wilms tumor Report of longest-known survivor and guidelines for conservative management

Abstract

In a one-year-old black male patient a classic Wilms tumor of the left kidney was treated with left nephrectomy, chemotherapy, and irradiation to the entire abdomen. Two years later a mature Wilms tumor, consisting predominantly of rhabdomyomatous elements, was discovered in the right kidney. Although a second course of chemotherapy was given and 2,000 rad were delivered to the right flank, the mass continued to enlarge with progressive deterioration of renal function. A nephron-sparing procedure was performed, in which a 3,400-g tumor measuring 19 cm × 16 cm × 9 cm was enucleated from the right kidney without compromise to the remaining normal tissue. Pathologic examination of the surgical specimen revealed a mature Wilms tumor with a malignant anaplastic sarcoma arising in the central portion. Currently, he is disease free with normal renal function more than twenty years after diagnosis of the metachronous bilateral Wilms tumor. We believe he is the longest known surviving metachronous Wilms tumor patient and emphasizes the importance of conservative, meticulous surgery in the management of both unilateral and bilateral Wilms tumors. The role of multimodal therapy (chemotherapy, irradiation, and surgery) as well as the current guidelines for the management of bilateral Wilms tumors as proposed by the National Wilms Tumor Study are reviewed.