Huang Kuo-How, Shun Chia-Tung, Huang Shin-Yi, Yu Hong-Jeng, Chueh Shih-Chieh, Chen Jun
Department of Urology, National Taiwan University Hospital, Taipei, Taiwan.
J Formos Med Assoc. 2006 Dec;105(12):1008-12. doi: 10.1016/S0929-6646(09)60285-0.
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 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 is possible if complete resection is performed at an early stage.
原发性泌尿系统原始神经外胚层肿瘤（PNET）是一种罕见疾病，具有侵袭性且预后较差。我们分析了1984年至2004年间我院收治的851例泌尿系统恶性肿瘤病例。仅发现3例（0.035%）泌尿系统PNET病例。临床表现为胁腹痛和血尿。第一例是一名44岁男性，患有左肾PNET，接受了手辅助腹腔镜根治性肾切除术及辅助化疗。4年随访时未见肿瘤复发。第二例是一名75岁女性，患有右肾PNET并伴有下腔静脉（IVC）血栓形成，血栓延伸至右心房。患者接受了右根治性肾输尿管切除术及体外循环下的IVC血栓切除术。7个月后死于转移性疾病。第三例是一名45岁男性，患有左输尿管PNET。实施了左输尿管节段性切除术及部分膀胱切除术。7年后发现肿瘤复发。复发后1年患者死于播散性疾病。泌尿系统PNET似乎是一种侵袭性恶性肿瘤。如果早期进行完整切除，有可能实现长期生存。
