Purpose: We strove to characterize the clinicopathologic features of a
rare malignant tumor involving the urogenital system. Materials and M
ethods: Of 109 reported cases of desmoplastic small round-cell tumor,
25 have involved organs ordinarily evaluated and treated by urologists
. Experience based on these cases plus 2 from our files forms the basi
s of this review. Results: The term desmoplastic small round-cell tumo
r has been applied to a rare, highly malignant neoplasm that occurs mo
st often in the abdominal cavity and is described in the nonurological
literature. This cancer has distinctive histological, immunohistochem
ical and ultrastructural features. Several reports suggest a character
istic genetic abnormality. All reported patients who have tumors invol
ving the genitourinary structures have been treated with chemotherapy,
but prognosis has been poor. Conclusions: The literature on desmoplas
tic small round-cell tumor indicates that a significant percentage of
these rare malignancies involve genitourinary organs. In most cases, t
umors have been unresectable surgically and treatment has been multidr
ug chemotherapy. Response to treatment has been poor, with only 3 of 2
5 determinate cases free of disease when reported.