A 60-year-old woman was admitted to our hospital with pain in the left flank. Retrograde pyelography, computed tomographic scan, and magnetic resonance imaging demonstrated left hydronephrosis due to a 7 cm retroperitoneal mass involving the left ureter. Left nephroureterectomy and partial resection of the mesentery revealed a primary ureteral leiomyosarcoma. Three months postoperatively, the patient received systemic chemotherapy (CYVADIC; cyclophosphamide, vincristine, adriamycin and DTIC) for a recurrent tumor. Two courses of chemotherapy reduced the tumor by nearly 60%. Then we performed surgery in an attempt to resect the residual disease. However, the tumor continued to progress and the patient died approximately one year after diagnosis.
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