Abstract
BACKGROUND: Wilms tumor (WT) is the most common pediatric renal malignancy, but carries a significantly worse prognosis in the adult population. Late recurrence (LR) of a primary WT, defined as disease recurrence five or more years after initial diagnosis, is rare and does not have standardized treatment regimens outside the pediatric population. In this paper we report a LR 32 years after diagnosis, the longest documented disease-free interval ever described. CASE PRESENTATION: A one-year-old female patient was diagnosed with bilateral, favorable-histology WT and treated with chemotherapy. Biopsies of residual masses after treatment confirmed benign tissue, and she was observed with serial imaging for seventeen years without evidence of recurrence. Thirty-two years after initial diagnosis, she presented with gross hematuria and right-sided abdominal pain. Biopsy demonstrated of the left renal mass was consistent with a LR of WT. She received neoadjuvant chemotherapy followed by left partial nephrectomy, right radical nephrectomy, and adjuvant radiation therapy and remains cancer free without any evidence of tumor recurrence. CONCLUSION: This case of a 32-year treatment-free interval prior to LR is the longest such case in the medical literature. This case highlights the importance of multidisciplinary management in this rare clinical scenario of LR WT.