Primary ureteral adenocarcinoma is an infrequent histological type of urinary neoplasm. Many authors consider intestinal metaplasia the pivotal event of the pathogenetic process, whether it occurs on a pre-existing urothelial carcinoma or on a normal urothelium. Diagnosis is essentially based on case history and clinical findings (hematuria and pain) and on diagnostic imaging. Treatment is surgical and the ideal procedure is nephroureterectomy with excision of a bladder margin adjacent to the ureteral opening and ispilateral para-aortoiliac lymphadenectomy. A 76-year-old man with primary adenocarcinoma of the ureter case is reported.
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