Abstract
The management of gastrointestinal and pancreatic (GEP) neuroendocrine tumors (NETs) has evolved over the recent decade. Primary renal NETs are extremely rare as neuroendocrine cells are not recognized in the normal renal parenchyma. We report a case of primary renal NET characterized by the initial diagnostic challenges. Recurrent and metastatic disease was managed along the lines of management of GEP-NETs, leading to prolonged progression-free survival.