Gehle Daniel B, Morrison Zachary D, Halepota Huma F, Kumar Akshita, Gwaltney Clark, Krasin Matthew J, Graetz Dylan E, Santiago Teresa, Boston Umar S, Davidoff Andrew M, Murphy Andrew J
Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
Department of Surgery, University of Tennessee Health Science Center, Memphis, TN 38163, USA.
Children (Basel). 2024 Jul 25;11(8):896. doi: 10.3390/children11080896.
Wilms tumor (WT) is the most common kidney tumor in pediatric patients. Intravascular extension of WT above the level of the renal veins is a rare manifestation that complicates surgical management. Patients with intravascular extension are frequently asymptomatic at diagnosis, and tumor thrombus extension is usually diagnosed by imaging. Neoadjuvant chemotherapy is indicated for thrombus extension above the level of the hepatic veins and often leads to thrombus regression, obviating the need for cardiopulmonary bypass in cases of cardiac thrombus at diagnosis. In cases of tumor extension to the retrohepatic cava, neoadjuvant therapy is not strictly indicated, but it may facilitate the regression of tumor thrombi, making resection safer. Hepatic vascular isolation and cardiopulmonary bypass increase the risk of bleeding and other complications when utilized for tumor thrombectomy. Fortunately, WT patients with vena caval with or with intracardiac extension have similar overall and event-free survival when compared to patients with WT without intravascular extension when thrombectomy is successfully performed. Still, patients with metastatic disease at presentation or unfavorable histology suffer relatively poor outcomes. Dedicated pediatric surgical oncology and pediatric cardiothoracic surgery teams, in conjunction with multimodal therapy directed by a multidisciplinary team, are preferred for optimized outcomes in this patient population.
肾母细胞瘤（WT）是儿科患者中最常见的肾脏肿瘤。WT在肾静脉水平以上的血管内扩展是一种罕见的表现，会使手术管理复杂化。血管内扩展的患者在诊断时通常没有症状，肿瘤血栓扩展通常通过影像学诊断。对于肝静脉水平以上的血栓扩展，新辅助化疗是必要的，并且常常导致血栓消退，从而避免在诊断时出现心脏血栓的情况下进行体外循环。在肿瘤扩展至肝后下腔静脉的情况下，新辅助治疗并非严格必需，但它可能有助于肿瘤血栓的消退，使切除术更安全。当用于肿瘤血栓切除术时，肝血管隔离和体外循环会增加出血及其他并发症的风险。幸运的是，与没有血管内扩展的WT患者相比，成功进行血栓切除术后，有腔静脉或心脏内扩展的WT患者的总生存率和无事件生存率相似。然而，就诊时伴有转移性疾病或组织学不良的患者预后相对较差。为了使该患者群体获得最佳治疗效果，首选专业的儿科外科肿瘤学和儿科心胸外科团队，并结合多学科团队指导的多模式治疗。
