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Neuroendocrine tumors (NETs) of the ureter are rare, with less than 40 cases described in the literature. A majority of tumors described are poorly differentiated tumors with a poor prognosis. We present the case of a moderately differentiated atypical carcinoid NET of the ureter with a good postoperative outcome. A literature review was also performed to identify similar cases to compare their management and postoperative outcomes.

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