Primary Primitive Neuroectodermal Tumor of the Urinary Tract

Primary primitive neuroectodermal tumor (PNET) of the urinary tract is a rare disease with aggressive behavior and poor prognosis. We analyzed 851 cases of urinary tract malignancies in our hospital between 1984 and 2004. Only three (0.035%) cases with PNET of the urinary tract were identified. Presenting symptoms included flank pain and hematuria. The first case was a 44-year-old man with left renal PNET who underwent hand-assisted laparoscopic radical nephrectomy and adjuvant chemotherapy. There was no recurrent tumor at the 4-year follow-up. The second case was a 75-year-old woman with right renal PNET with inferior vena cava (IVC) thrombosis extending to the right atrium. The patient underwent right radical nephroureterec-tomy and IVC thrombectomy with cardiopulmonary bypass. She died of metastatic disease 7 months later. The third case was a 45-year-old man with left ureteral PNET. Left ureteral segmental resection and partial cystectomy were performed. Tumor recurrence was noted 7 years later. The patient died of disseminated disease 1 year after the discovery of recurrence. Urinary tract PNET appears to be an aggressive malignancy. Long-term survival is possible if complete resection is performed at an early stage.