Wilms' tumor (nephroblastoma) is the most common renal tumor in children. It is uncommon in adolescent and skeletal metastasis at the time of presentation is even rare. The present case is a rare monophasic blastemal variant of Wilms' tumor presenting with multiple skeletal metastases in a young female. We report a case of 23-year-old female presented with severe low backache and colicky right loin pain of 1 month and constant dull aching left upper arm pain of 20 days duration. Contrast-enhanced computed tomography (CECT) abdomen revealed heterogeneously enhancing soft tissue density mass with central nonenhancing areas arising from the upper pole of the right kidney. Whole body 18F-Sodium Fluoride (NaF) Positron emission tomography-computed tomography (PET/CT) bone scintigraphy revealed multiple skeletal metastases. The patient underwent right radical nephrectomy and the tumor was confirmed as adult Wilms' tumor by histopathology and immunohistochemistry. Skeletal metastasis may be the first presentation in a case of adolescent Wilms' tumor, and this possibility should be considered when an adolescent patient presents with flank pain and a renal mass. The clinical course of adult Wilms' tumor with blastemal component is very aggressive with high chances of recurrence and mortality.
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