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Cancers of the ureter and renal pelvis are rare and occur more commonly in patients who have bladder cancer, Balkan nephropathy, or Lynch syndrome, who smoke, or who have a long history of analgesic abuse. The majority are urothelial cell carcinomas. Gross or microscopic hematuria is present in most patients; flank pain secondary to bleeding and obstruction occurs less commonly. As with bladder cancers, urinary cytology is often positive in high-grade cancers. The most common signs identified at the time of CT or intravenous urography include an intraluminal filling defect, unilateral nonvisualization of the collecting system, and hydronephrosis. Ureteral and renal pelvic tumors must be differentiated from calculi, blood clots, papillary necrosis, or inflammatory and infectious lesions. Upper urinary tract lesions are accessible for diagnostic biopsy, fulguration, or resection using a ureteroscope. Treatment is based on the site, size, grade, depth of penetration, and number of cancers present. Endoscopic resection may be indicated in patients with limited renal function or focal, low-grade, cancers. Chemoablation with a mitomycin infused gel can be performed in very select patient with low-grade upper tract lesions. Most high-grade and high-volume cancers are excised with robotic, laparoscopic, or open nephroureterectomy (renal pelvic and upper ureteral lesions) or segmental excision of the ureter (distal ureteral lesions). A phase 3, randomized trial demonstrated significantly improved 3-year disease-free survival with dual agent adjuvant systemic chemotherapy after nephroureterectomy. Like with urothelial bladder cancers, use of chemotherapy prior to surgery may also improve outcomes and remains under investigation.

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