A 30-year-old male underwent radical nephrectomy for a right renal tumor 15
cm in diameter. On microscopic examination of initial 17 sections. the tum
or consisted of pleomorphic giant cells and spindle neoplastic cells. There
was no carcinomatous component. Immunohistochemically, the neoplastic cell
s were negative for keratin and epithelial membrane antigen but positive fo
r vimentin. The giant cells were also scatteringly, weakly positive for myo
globin. At that time a diagnosis of rhabdomyosarcoma of the kidney was made
. However, further microscopic examination of another eight sections reveal
ed small areas of clear cell-type renal cell carcinoma (RCC) which transite
d to sarcomatous components and led to a diagnosis of sarcomatoid RCC, The
patient underwent three cycles of adjuvant chemotherapy. He has been free o
f the disease for 14 months after nephrectomy.