Journal of the Formosan Medical Association
Volume 105, Issue 12 , Pages 1008-1012, 2006

Primary Primitive Neuroectodermal Tumor of the Urinary Tract

  • Chia-Tung Shun

      Affiliations

    • Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan
    • ,
  • Shin-Yi Huang

      Affiliations

    • Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan

Department of Urology, National Taiwan University Hospital, Taipei, Taiwan

Received 6 October 2005; received in revised form 25 November 2005; accepted 7 February 2006.

Article Outline

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.

Key Words:  immunohistochemical staining , primitive neuroectodermal tumor , urinary tract

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PII: S0929-6646(09)60285-0

doi:10.1016/S0929-6646(09)60285-0

Journal of the Formosan Medical Association
Volume 105, Issue 12 , Pages 1008-1012, 2006

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