Urogenital involvement by desmoplastic small round-cell tumor

ABSTRACTPurpose: We strove to characterize the clinicopathologic features of a rare malignant tumor involving the urogenital system.Materials and Methods: 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 basis of this review.Results: The term desmoplastic small round-cell tumor has been applied to a rare, highly malignant neoplasm that occurs most often in the abdominal cavity and is described in the nonurological literature. This cancer has distinctive histological, immunohistochemical and ultrastructural features. Several reports suggest a characteristics genetic abnormality. All reported patients who have tumors involving the genitourinary structures have been treated with chemotherapy, but prognosis has been poor.Conclusions: The literature on desmoplastic small round-cell tumor indicates that a significant percentage of these rare malignancies involve ge...