Primitive Neuroectodermal Tumor (PNET) ofKidney -A Rare Entity

Primitive  neuroectodermal  tumor  (PNET)  of  kidney  is  an  extremely  rare  renal  neoplasm  with only about fifty reported cases in literature.  Presumably,of neural crest origin, these tumors behave  aggressively  and  carry  a  poor prognosis.  We  report  a  case  of  22-year  old  female  patient complaining of left loin pain with recurrent hematuria for last 3 months. On clinical examination, the abdomen was soft and no palpable mass was felt. She underwent ultrasonography and com puted  tomography, which revealed a left renal mass. A left radical nephrectomy was performed. Histopathological examination of the  nephrectomy  specimen showed features of primitive  neu roectodermal  tumor  arising  from  left  kidney,  which was  confirmed  by  immunohistochemistry (IHC). The patient was treated with post-nephrectomy chemotherapy  and was  symptom-free at six-month follow-up.