Renal primitive neuroectodermal tumour in childhood: Case report and review of literature.

Primitive neuroectodermal tumour (PNET) is presumed to be of neural crest origin, mostly presenting as bone or soft tissue masses. It usually occurs in the trunk or axial skeleton; while renal PNET is considered an extremely rare tumour. We report a case of 11-year-old male who presented with right flank pain and gross hematuria after suffering blunt trauma. During investigations, he was found to have a large renal mass on computed tomography. He underwent a right radical nephrectomy where the pathology report showed PNET of the kidney. The patient received chemotherapy afterwards. Despite the chemotherapy, he had a local tumour recurrence 3 months after and continued to deteriorate and developed distant metastasis. Primitive neuroectodermal tumour of the kidney is a distinct and rare entity. It is very aggressive, with a poor survival despite combined modality treatment.