Department of Paediatric Surgery, Nottingham University Hospitals, Nottingham, UK.
Department of Paediatric Surgery, Leeds Teaching Hospitals, Leeds, UK.
Pediatr Blood Cancer. 2019 Nov;66(11):e27930. doi: 10.1002/pbc.27930. Epub 2019 Jul 24.
Preoperative chemotherapy is recommended for children with Wilms tumour with intravascular extension. Extended chemotherapy may improve resectability, but increase tumour adherence to vascular endothelium, precluding complete resection. To evaluate the optimal length of preoperative treatment, we report a two-part review comprising systematic review of the literature and investigation of patients treated in the International Society of Paediatric Oncology (SIOP) WT 2001 trial.
Studies were identified using Medline and Embase databases from 1996 to present. English language titles reporting management of intravascular Wilms tumour were analysed. Patients with Wilms tumour and thrombus were identified from the SIOP WT 2001 trial. Overall survival (OS) and event-free survival (EFS), tumour regression, completeness of resection and cavectomy were investigated.
The search retrieved 43 articles documenting 498 children. Note that 72% of the patients received neoadjuvant chemotherapy: 101 received standard course (4-6 weeks, standard course neoadjuvant chemotherapy [StC]) and 62 extended course (> 6 weeks, extended course neoadjuvant chemotherapy [EC]). There was no significant difference between the groups in terms of thrombus regression or completeness of resection. EFS was greater in the StC group (78 vs 54%; P = .04). Of 4511 patients registered in the SIOP WT 2001 trial, 166 had thrombus. Note that 97% of the patients received neoadjuvant chemotherapy: 63 StC and 67 EC. There was no significant difference between the groups with regard to tumour regression, complete resection, or cavectomy. Survival was significantly higher in those receiving StC than EC (OS: 95% vs 82%, P = .025; EFS: 88% vs 72%, P = .047).
There is no evidence that prolonged courses of neoadjuvant chemotherapy beyond the recommended protocols confer any additional benefit in treating intravascular extension of Wilms tumour.
对于有血管内延伸的威尔姆斯瘤患儿，建议进行术前化疗。延长化疗可能会提高可切除性，但会增加肿瘤对血管内皮的黏附性，从而妨碍完全切除。为了评估最佳的术前治疗长度，我们报告了一个由两部分组成的综述，包括对文献的系统综述和对国际小儿肿瘤学会（SIOP）WT 2001 试验中治疗的患者的调查。
使用 Medline 和 Embase 数据库从 1996 年到现在对研究进行了识别。分析了报告血管内威尔姆斯瘤治疗管理的英文标题的文章。从 SIOP WT 2001 试验中确定了患有威尔姆斯瘤和血栓的患者。调查了总生存率（OS）和无事件生存率（EFS）、肿瘤消退、切除的完整性和腔静脉切除术。
搜索共检索到 43 篇文献，其中 498 例患儿。请注意，72%的患者接受了新辅助化疗：101 例接受标准疗程（4-6 周，标准疗程新辅助化疗[StC]），62 例接受延长疗程（>6 周，延长疗程新辅助化疗[EC]）。两组在血栓消退或切除的完整性方面没有显著差异。StC 组的 EFS 更高（78% vs 54%；P=0.04）。在 SIOP WT 2001 试验中登记的 4511 例患者中，有 166 例有血栓。请注意，97%的患者接受了新辅助化疗：63 例接受 StC，67 例接受 EC。两组在肿瘤消退、完全切除或腔静脉切除术方面没有显著差异。与 EC 组相比，接受 StC 的患者生存率显著更高（OS：95% vs 82%，P=0.025；EFS：88% vs 72%，P=0.047）。
没有证据表明，超出推荐方案的延长新辅助化疗疗程在治疗威尔姆斯瘤血管内延伸方面没有任何额外益处。
