外科治疗小儿肾母细胞瘤伴瘤栓11例报告

Surgical management of Wilms＇ tumor with intravascular thrombus in children

目的探讨小儿肾母细胞瘤伴瘤栓的治疗方法，总结治疗体会和经验。方法回顾性分析2000年1月至2012年6月复旦大学附属儿科医院外科收治的11例小儿肾母细胞瘤伴瘤栓的临床资料，包括临床表现、影像学资料、手术方法、化疗方案和疗效。其中，男7例，女4例；平均年龄（3．4±2．1）岁；主要临床表现为腹部包块和（或）血尿，肺转移1例；术前经彩色多普勒超声、CT和（或）MRI诊断肾脏肿瘤伴瘤栓。按Daum分型，本组工型瘤栓3例，Ⅱ型4例，Ⅲ型3例，Ⅳ型1例。所有病例均进行术前化疗，化疗前活检7例；化疗4～10周后行肾母细胞瘤切除术并取栓。其中，切开部分或全部肝下下腔静脉（IVC）取栓7例，非心肺旁路（CPB）辅助下全肝阻断切开整个IVC取栓3例，CPB和深低温心脏停跳（DHcA）辅助下切开IVC及右心房取栓1例。术后接受腹部放疗4例。结果本组11例患儿均顺利切除肾母细胞瘤并成功取栓，瘤栓长度2．5～13．0cm，平均（6．8±5．5）cml3例非CPB辅助下取栓的全肝阻断时间为13～20min。1例CPB＋DHCA辅助下取栓的CPB时间约为25min。无手术中死亡病例。术后病理检查证实为预后良好型（FH）肾母细胞瘤7例，预后不良型（uH）4例；7例瘤栓内有肿瘤细胞。术后随访0．8～9．5年，平均（3．4±2．7）年，无瘤生存9例，术后9个月复发死亡1例，失访1例。结论小儿肾母细胞瘤伴瘤栓的治疗是极具挑战性的工作。积极的术前化疗、及时进行肾母细胞瘤切除术和取栓是治疗小儿肾母细胞瘤伴瘤栓的根本方法。

Objective To review the outcomes and experiences of surgical management for Wilms＇ tumor with intravaseular thrombus in children. Methods From January 2000 to June 2012, 11 patients underwent treatment for Wilms＇ tumor with intracaval or atrial thrombus. The data were collected regarding clinical characteristics, chemotherapy, operative details, tumor and thrombus histology and long-term outcomes. Preoperative chemotherapy was administered in all patients and postoperative radiotherapy in 4 cases. There were intravaseular involvement type I （n = 3）, type Ⅱ （n = 4）, type Ⅲ （n = 3） and type Ⅳ （n = 1）. The procedures for excision of intra-vascular component included local cavotomy （n = 7）, extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass （CPB） （n = 3） and excision of cavo-atrial thrombus with CPB and deep hypothermia and circulatory arrest （DHCA） （n = 1）, Results There was no instance of intraoperative mortality. The block time of extensive infra-diaphragmatic cavotomy without CPB was 13-20 rain. CPB on cavotomy and atriotomy was 25 min. Tumor histology was either favorable （n = 7） or unfavorable （n = 4）. And thrombus contained malignant cells （n = 7）. The mean length of thrombus was （6. 8 ± 5. 5） （2. 5- 13. 0） cm. Ten patients were followed up for a mean period of 3. 4 ± 2. 7 （0. 8-9. 5） years. Nine patients remained disease-free, only 1 recurrent case died and 1 case was lost to follow-up. Conclusions Surgical management for Wilms＂ tumor with intravascular extension has remained technically challenging. The outcome of Wilms＂ tumor with intravascular extension in children depends on complete surgical excision with optimal chemotherapy.