儿童肾母细胞瘤合并下腔静脉及心房瘤栓1例并文献复习

Wilms Tumor with Inferior Vena Cava and Atrial Thrombus in Children: A Case Report and Literature Review

目的:探讨儿童肾母细胞瘤合并下腔静脉及心房瘤栓的诊断、治疗和预后。方法:回顾分析1例诊断为巨大肾母细胞瘤合并下腔静脉及心房瘤栓患儿的临床资料及诊疗过程,并对相关文献进展进行复习。结果:本例患儿为6岁男童,以腹部膨隆为主要表现,化疗过程中出现心悸、气促,CT提示左肾巨大肿瘤、心房及腔静脉内瘤栓,择期行一期手术,完整切除原发肿瘤后,于体外循环下切开右心房及下腔静脉摘除瘤栓。肿瘤大部分坏死,未能分型,术后病理报告示肾母细胞瘤,术后第2天开始化疗,化疗7个疗程后,放疗12次,随访8个月至今无瘤生存。结论:一期胸腹联合手术并结合化疗及放疗可以有效地治疗儿童肾母细胞瘤合并下腔静脉及心房瘤栓,延长生命,降低死亡率。

Objectives: To investigate the diagnosis, treatment, and follow-up of wilms tumor with cavoatrial tumor-thrombus in children. Methods: Review of the clinical data of a case diagnosed as giant wilms tumor with inferior vena cava and right heart extension with related literatures. Results: A 6-year-old boy initially presented with distended abdomen, and developed palpitation, short-winded during chemotherapy. A giant tumor was showed in left kidney with atrium and inferior vena cava thrombosis at CT. After the primary tumor complete resected of, the child was removed the inferior vena cava and atrial thrombus through thracic and abdominal approach under cardiopulmonary bypass (CPB) with deep hypothermia and circulatory arrest (DHCA). The tumor with nearly complete necrosis, failed to classify the histological type, was showed Wilms tumor by postoperative pathology. The chemotherapy was started on the next day after operation. Follow-up of 8 months, the boy is still free survival, Combined with Radiotherapy 12 times and 7 courses of chemotherapy after surgery. Conclusions: A thoracoabdominal surgery combined with chemotherapy and radiotherapy can effectively treat Wilms’ tumor with inferior vena cava and atrial thrombus in children, prolong life, and reduce mortality.