The authors present two cases of primary synovial sarcoma of the kidney. Bo
th patients had a mass in the upper part of the right kidney without any pr
imary extrarenal neoplastic lesions. Grossly, the tumors were soft to rubbe
ry masses measuring 5.5 cm and 5 cm in diameter, respectively. Histological
ly, both tumors were poorly differentiated synovial sarcoma. The lesions ex
hibited a hypercellular solid or lobular growth of round, oval, or short sp
indle cells in variably solid sheets, in intersecting fascicles, or in a ha
phazard fashion. Areas of solid aggregation or fascicles of the tumor cells
alternating with hypocellular myxoid tissues, together with areas displayi
ng a prominent hemangiopericytoma-like pattern, were found. Immunohistochem
ically, vimentin was diffusely positive and a few tumor cells were positive
fur cytokeratin, epithelial membrane antigen, and neurofilament. The tumor
cells were negative for S-100 protein, CD34, smooth muscle actin, and desm
in, whereas CD56 and CD99 were positive. In both cases, reverse transcripti
on-polymerase chain reaction using ribonucleic acid extracted from formalin
-fixed, paraffin-embedded tissues detected SYT-SSX2 fusion gene transcripts
, which are characteristic molecular findings of synovial sarcoma. One pati
ent died 10 months after diagnosis. These tumors are unique cases of primar
y synovial sarcoma of the kidney confirmed by molecular study.