肾母细胞瘤伴静脉瘤栓的治疗探讨

Treatment experiences of Wilm's tumor with vena cava thrombus

目的总结本院近6年来诊治的肾母细胞瘤伴静脉瘤栓病例资料,探讨此类患儿的治疗原则。方法回顾性分析2010年至2016年在本院诊断并接受系统治疗的肾母细胞瘤伴静脉瘤栓患儿临床资料,共12例。其中男性4例,女性8例(男女比例为1∶2)。平均首诊年龄3.94岁(最小1.5岁,最大7岁)。左侧5例,右侧7例。临床分期Ⅰ期5例,Ⅱ期4例,Ⅳ期3例(其中2例为肺转移,1例为肝脏和肺转移)。静脉瘤栓分型:Ⅰ型(下腔静脉的瘤栓在肾静脉水平以上,但距离小于2 cm)5例;Ⅱ型(瘤栓在肾静脉水平以上,距离超过2 cm,未超过肝门)2例;Ⅲ型(瘤栓超过肝门水平,但在横膈以下)2例;Ⅳ型(瘤栓超过横隔水平)3例。结果 12例患儿中,5例Ⅰ型瘤栓及1例Ⅱ型瘤栓患儿在明确诊断后即行肿瘤及瘤栓切除术,其余6例均在手术前接受化疗,肿瘤及瘤栓缩小后再行肿瘤及瘤栓切除术。5例瘤栓有残留。12例手术后均恢复良好,术后病理检查结果为肾母细胞瘤。经过术前化疗的病例中,瘤栓中均未见肿瘤细胞,仅为纤维组织及泡沫细胞。Ⅰ型瘤栓与静脉壁无粘连,Ⅱ至Ⅳ型瘤栓均下腔静脉壁有不同程度的粘连或浸润。全部患儿均完成方案中所有化疗程序及放疗。均于门诊定期随访0.5~6年,未见肿瘤复发。其中5例瘤栓残留病例在随访过程中未发现下腔静脉瘤栓有明显变化。结论①新辅助化疗对于此类患儿十分有必要;②根治手术中,不必过分追求瘤栓的完整切除,可以允许少量的瘤栓残留;③术后持续的规范化化疗和放疗是良好预后的保证。

Objective To explore the treatment and prognosis of Wilm＇s tumors with vena cava thrombus.Methods The clinical data were reviewed and analyzed for 12 patients of Wilm＇s tumors with vena cava thrombus and received systemic therapy from 2010 to 2016. Results There are 4 boys and 8 girls with an average diagnostic age of 3. 94（ 1. 5-7） years. The involved side was left（ n = 5） and right（ n = 7）. And the stages wereⅡ（ n =5）,Ⅲ（ n =4） and Ⅳ（ n = 3）. The thrombus level wasⅠ（ n = 5）,Ⅱ（ n = 2）,Ⅲ（ n =2） and Ⅳ（ n = 3）.Five level I and 1 level II patients underwent immediate surgery for total thrombus resection. Another 6 patients received preoperative neoadjuvant chemotherapy and 5 of them had residual thrombus. All patients recovered well postoperatively and pathology confirmed Wilm＇s tumor. Tumor cells were absent in thrombus of patients on neoadjuvant chemotherapy. All patients completed complete chemoradiotherapy. There was no recurrence during a follow-up period of 0. 5 ～ 6 years. The residual thrombus of 5 patients showed no obvious change. Conclusion Neoadjuvant chemotherapy is required for patients with vena cava thrombus. Minimal residual of thrombus is accpetable. Systemic chemotherapy and radiotherapy after surgery is essential for achieving an excellent prognosis for Wilm＇s tumor with vena cava thrombus.