“…Unlike carcinoid tumors arising in the gastrointestinal tract and lung, a definitive cell of origin within the renal parenchyma has not been identified [6][7][8]. Proposed theories for the origin of primary renal carcinoid tumors include derivation from neural crest cells entrapped within the metanephros during embryogenesis [9], neuroendocrine differentiation of a primitive totipotential cell [10,11], and hyperplasia of preexisting neuroendocrine cells within metaplastic or teratomatous epithelium [9,[11][12][13][14]. A review by Romero et al [2] demonstrated an association between renal carcinoid tumors and horseshoe kidneys, renal teratomas, and polycystic kidney disease in 18%, 14%, and 2% of reported cases, respectively [15].…”