累及泌尿系统的外周原始神经外胚层肿瘤

目的探讨累及泌尿系统的外周原始神经外胚层肿瘤(pPNET)的临床特点、免疫组化特点和诊断标准，提高pPNET的诊治水平。方法回顾分析7例累及泌尿系统的pPNET患者临床资料，其中6例行肿瘤根治性切除术，1例前列腺pPNET术中发现肿瘤已侵犯膀胱颈部和直肠前壁，仅行活检术。结果7例术后病理和免疫组化检查均诊断为pPNET。围手术期无患者死亡，7例患者随访4～26个月，平均12个月，1例术后5个月死于肿瘤远处转移和肾功能衰竭。结论泌尿系统pPNET是一种好发于年轻男性的恶性肿瘤，病变进展迅速，确诊依靠病理，预后较差，但发病早期根治性手术仍有较大的治疗意义。

Peripheral primitive neuroectodermal tumor involving urinary system

Objective To investigate the clinical features,pathological and immunohistochemical characteristics of peripheral primitive neuroectodermal tumor(pPNET) which involves urinary organs,and to improve the diagnosis and treatment level of this disease. Methods The clinical data of 7 male patients (age range,16-63 years) with pPNET involving urinary organs were retrospectively analyzed.Radical resection of the tumor was performed in 6 of the 7 patients.Invasion to the bladder neck and the anterior wall of the rectum was found during the operation in the remaining 1 patient with pPNET of the prostate.Therefore,only biopsy was performed in this case. Results The post-operative pathologic and immunohistochemical examinations confirmed the pPNET diagnosis.No death or serious morbidity developed during the operation.Six patients survived during the follow-up (range,4-26 months;mean,12 months).One patient died of systemic metastasis and renal failure 5 months after the operation. Conclusions pPNET is a malignant tumor usually occurring in young males and the tumor grows fast.The diagnosis of the disease depends on the pathologic examination.Despite of the poor prognosis of pPNET,radical resection is treatment of choice for early stage cases.