Lall Anupam, Pritchard-Jones Kathy, Walker Jenny, Hutton Caroline, Stevens Suzanne, Azmy Amir, Carachi Robert
Department of Pediatric Surgery, Royal Manchester Children's Hospital, M27 4HA Manchester, UK.
J Pediatr Surg. 2006 Feb;41(2):382-7. doi: 10.1016/j.jpedsurg.2005.11.016.
BACKGROUND/PURPOSE: To define the clinical characteristics and surgical management of intracaval involvement in patients enrolled in the UKW3 trial (1991-2001), which recommended elective preoperative chemotherapy for such cases.
Cases were identified from preoperative imaging and surgical trial forms. These asked specific questions about whether the surgeon suspected intracaval extension at diagnosis or found it at nephrectomy. For tumors with Wilms' histology, original case notes were examined.
Of 842 patients registered in UKW3, 730 (87%) had Wilms' tumor. Among them, 59 (8.1%) had evidence of intracaval extension, either documented at diagnosis (53) or found unexpectedly at nephrectomy (6). Intracaval extension was also seen in tumors of other histology. The level of thrombus was intraatrial (10), suprahepatic (9), retrohepatic (8), infrahepatic (26), and unknown (6). The median age at diagnosis was 3.75 years compared to 2.97 years in patients without inferior vena cava thrombus (P < .0001). Fifty-two of 59 received preoperative chemotherapy. Thirty-one (52%) needed cavotomy, and 3 (30%) with intraatrial extension required cardiopulmonary bypass. The commonest operative complication was significant hemorrhage and resulted in mortality in 3 cases.
Preoperative chemotherapy is a useful adjunct to shrink the tumor and thrombus. This reduces the requirement for cavotomy and cardiopulmonary bypass. Intraoperative hemorrhage remains a significant cause of operative morbidity and mortality.
背景/目的：明确参加UKW3试验（1991 - 2001年）的患者腔静脉受累的临床特征及手术治疗方法，该试验推荐对此类病例进行选择性术前化疗。
从术前影像学检查和手术试验表格中识别病例。这些表格询问了外科医生在诊断时是否怀疑腔静脉扩展或在肾切除术中是否发现腔静脉扩展等具体问题。对于具有威尔姆斯瘤组织学特征的肿瘤，检查原始病例记录。
在UKW3登记的842例患者中，730例（87%）患有威尔姆斯瘤。其中，59例（8.1%）有腔静脉扩展的证据，要么在诊断时记录（53例），要么在肾切除术中意外发现（6例）。其他组织学类型的肿瘤中也可见腔静脉扩展。血栓水平为心房内（10例）、肝上（9例）、肝后（8例）、肝下（26例）和不明（6例）。诊断时的中位年龄为3.75岁，而无下腔静脉血栓的患者为2.97岁（P <.0001）。59例中有52例接受了术前化疗。31例（52%）需要进行腔静脉切开术，3例（30%）有心房内扩展的患者需要体外循环。最常见的手术并发症是严重出血，3例导致死亡。
术前化疗是缩小肿瘤和血栓的有效辅助手段。这减少了腔静脉切开术和体外循环的需求。术中出血仍然是手术 morbidity 和 mortality 的重要原因。
