A 34-year-old man presented with a flu-like syndrome and was found to have a right upper quadrant mass on examination. The CT scan showed a large mass originating out of the right kidney with multiple liver lesions.
The inferior vena cava and right renal vein were compressed and displaced without evidence of thrombosis. Fine needle aspiration of the mass showed a malignant small round blue cell tumor. The differential included monophasic Wilm's tumor, embryonal rhabdomyosarcoma and primitive neuroectodermal tumor. Less likely were desmoplastic small round blue cell tumor and monophasic synovial sarcoma.
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