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目的:探讨肾脏原始神经外胚层肿瘤(rPNET)合并下腔静脉癌栓的临床表现、影像检查、病理特点、治疗方法及预后。方法:回顾性分析我院2008年1月~2014年1月rPNET合并下腔静脉癌栓的5例患者的临床资料,包括临床表现、术前诊断、手术方法、病理结果和患者的预后。结果:5例患者术中均成功施行经腹肾癌根治性切除及癌栓清除术,手术时间平均244(190~310)min;术中平均出血量940(600~1 400)ml;术后平均输血800(400~1 400)ml;术中平均阻断血流时间12(10~20)min;术后平均住院时间15(12~24)d。平均随访24(6~60)个月,2例死于肾癌转移,1例发现远处转移,其余2例为无瘤生存。结论:rPNET合并静脉癌栓在临床上极为少见,治疗以根治性手术为首选,同时合并放化疗等综合治疗可提高生存率。
Objective: To investigate the clinical manifestations, imaging, pathological features, treatment and prognosis of nephrotic primitive neuroectodermal tumors (rPNET) with inferior vena cava tumor thrombus. Methods: The clinical data of 5 patients with rPNET complicated with IVC tumor thrombus in our hospital from January 2008 to January 2014 were retrospectively analyzed. The clinical data including clinical manifestations, preoperative diagnosis, surgical methods, pathological findings and prognosis of the patients were included. Results: Five patients underwent radical resection of renal cell carcinoma and tumor thrombus removal during operation. The average operation time was 244 (190-310) min. The average blood loss during operation was 940 (600-1 400) ml. After operation The mean blood transfusion was 800 (400-1 400) ml. The average intraoperative blood-block time was 12 (10-20) min. The average postoperative hospital stay was 15 (12-24) days. The average follow-up was 24 months (6 ~ 60 months). Two cases died of renal cell carcinoma metastasis, one case had distant metastasis, and the other two cases were tumor-free survival. Conclusions: rPNET combined with venous thrombosis is extremely rare in clinical practice. Radical surgery is the first choice of treatment. Combined with radiotherapy and chemotherapy can improve the survival rate.