Department of Pediatric Hematology Oncology, Ankara Children's Hematology Oncology Training and Research Hospital, Altındağ, Ankara, Turkey.
Transl Pediatr. 2014 Jan;3(1):29-33. doi: 10.3978/j.issn.2224-4336.2014.01.03.
Wilms tumor (WT) is one of the most common solid tumors in children. It is the second most common extracranial solid tumor after neuroblastoma. WT has a strong tendency to invade blood vessels in the form of tumor thrombus, into the renal veins, and inferior vena cava and even into the right atrium. Extension of tumor thrombus along to the renal vein into the inferior vena cava occurs in 4-10% of all patients. Tumor thrombus extending to the right atrium is less reported as 0.7-1%. WT with renal vein thrombus has been reported to be more common in the right kidney because of the shorter right renal vein. Most patients with tumor thrombus are asymptomatic and diagnosis is only made on imaging investigations. Several imaging modalities including computed tomography (CT), magnetic resonance imaging (MRI) and Doppler ultrasonography (USG) can demonstrate intravascular tumor thrombus before surgery. In addition to CT and MRI, Doppler USG is reliable in demonstrating the presence and extent of inferior vena cava tumor thrombus. The management of WT with tumor thrombus is determined by multiple factors such as extent of tumor thrombus, chemotherapy response of the tumor. Now, it is generally recommended to use preoperative chemotherapy to a patient presenting with intravascular tumor thrombus. This approach is helpful to decrease the extent of the vascular thrombus which facilitates surgical excision. Most intracaval and intraatrial thrombi in WT show a response to chemotherapy. Neoadjuvant chemotherapy causes tumor regression in nearly half of the patients. Most of them can be managed without the need for cardiac bypass surgery. The decision of initial surgery or preoperative chemotherapy should be carefully determined on every case. Primary surgery would only be indicated in a patient who is unstable because of thrombus that might dislodge and cause acute symptoms. Presence of tumor thrombus in WT needs for multidisciplinary care including pediatric oncologists, pediatric surgeons, and pediatric cardiac surgeons.
威尔姆斯瘤（WT）是儿童中最常见的实体瘤之一。它是仅次于神经母细胞瘤的第二种最常见的颅外实体瘤。WT 有一种很强的倾向，以肿瘤血栓的形式侵犯血管，进入肾静脉、下腔静脉，甚至右心房。肿瘤血栓沿肾静脉延伸至下腔静脉在所有患者中的发生率为 4-10%。肿瘤血栓延伸至右心房的报道较少，为 0.7-1%。由于右肾静脉较短，右肾的 WT 更常伴有静脉血栓。大多数有血栓的患者无症状，仅在影像学检查中诊断。几种影像学方法，包括计算机断层扫描（CT）、磁共振成像（MRI）和多普勒超声（USG），可在手术前显示血管内肿瘤血栓。除 CT 和 MRI 外，多普勒 USG 还可可靠地显示下腔静脉肿瘤血栓的存在和范围。WT 伴肿瘤血栓的治疗取决于多个因素，如肿瘤血栓的范围、肿瘤的化疗反应。现在，一般建议对有血管内肿瘤血栓的患者进行术前化疗。这种方法有助于减少血管血栓的范围，便于手术切除。WT 中的大多数腔静脉和心房内血栓对化疗有反应。新辅助化疗使近一半的患者肿瘤缩小。其中大多数患者无需心脏旁路手术即可得到治疗。初始手术或术前化疗的决定应根据每个病例仔细确定。只有在因血栓可能脱落并引起急性症状而不稳定的患者中才需要进行原发性手术。WT 存在肿瘤血栓需要多学科的护理，包括儿科肿瘤学家、儿科外科医生和儿科心脏外科医生。
