Benign fibromatous tumor (fibroma) of the urogenital tract is a distin
ctive pathologic entity occurring in the testis, paratesticular struct
ures and renal peripelvis. We report a well-circumscribed tumor replac
ing the cortex and the medulla of more than half of the upper kidney,
radiographically thought to be renal cell carcinoma. Histologically, t
he tumor was characterized by a variable cellularity and was composed
of bland spindle-shaped cells loosely dispersed in a fibromyxoid to de
nsely fibrous stroma in which calcifications and chronic inflammation
were not observed. Immunocytochemistry showed that cells were positive
for vimentin and, only focally, stained positive for desmin and alpha
-smooth muscle actin. Differential diagnosis included a wide spectrum
of benign and malignant spindle cell tumors. The clinicopathologic fea
tures were consistent with benign fibromatous tumor (fibroma) of the k
idney. To our knowledge, this is the first case of benign fibromatous
tumor (fibroma) of the kidney reported in the English literature.