Primary primitive neuroectodermal tumor of the urinary tract
Journal
Journal of the Formosan Medical Association
Journal Volume
105
Journal Issue
12
Pages
1008-1012
Date Issued
2006
Author(s)
Abstract
Primary primitive neuroectodermal tumor (PNET) of 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 fight renal PNET with inferior vena cava (IVC) thrombosis extending to the right atrium. The patient underwent fight radical nephroureterectomy 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 if complete resection is performed at an early stage. ? 2006 Elsevier & Formosan Medical Association.
SDGs
Other Subjects
CD99 antigen; cyclophosphamide; docetaxel; doxorubicin; etoposide; ifosfamide; imatinib; mesna; neuron specific enolase; protein S 100; topotecan; transcription factor Fli 1; vincristine; adjuvant therapy; adult; aged; article; cancer adjuvant therapy; cancer mortality; cancer palliative therapy; cancer radiotherapy; cancer recurrence; cancer staging; cancer survival; case report; cavography; clinical feature; combination chemotherapy; computer assisted tomography; cystectomy; cystoscopy; disseminated cancer; female; flank pain; follow up; hematuria; histopathology; human; immunohistochemistry; inferior cava vein; laparoscopic surgery; laser surgery; male; metastasis; multiple cycle treatment; nephrectomy; nephroureterectomy; neuroectoderm tumor; nuclear magnetic resonance imaging; prognosis; thrombectomy; transurethral resection; ureteroneocystostomy; urinary tract cancer; vein thrombosis
Publisher
Scientific Communications International Ltd
Type
journal article