Surgery of cavoatrial tumor thrombus in nephroblastoma: A report of the SIOP/GPOH study

Abstract

Our report suggests that Wilms tumor extending to the inferior vena cava and the right atrium is technical challenging, but with adequate preoperative diagnosis and a multidisciplinary surgical approach including cardiopulmonary bypass and hypothermia, the prognosis is favorable.

Intracaval and intracardiac extension of Wilms' tumor: the influence of preoperative chemotherapy on surgical morbidity

“…The complications incidence rate in NWTS-3 is 43% (14,15). The SIOP/ GPOH group reports 18.18% of complications in 33 children with Wilms' tumor and thrombus extension, 29/33 submitted to preoperative chemotherapy (16). The UKW3 trial reports 13.6% of hemorrhagic complications, including 3 deaths (17).…”

Strategy for management of retroperitoneal tumors with caval tumor thrombus

“…Although previously reported in adults [10][11][12], intravascular extension with CCSK is rare [13], comprising only 2% of cases of tumor thrombus in the NWTS-4 [14]. However, the incidence of tumor thrombus among all cases of CCSK (6%) is similar to the incidence in Wilms' tumor (3-8%) [2,4]. IVC tumor thrombus has also been reported with pediatric renal cell carcinoma and malignant rhabdoid tumor [4].…”

“…Intravascular invasion occurs in 3-8% of patients with pediatric renal tumors [2][3][4] with Wilms' tumor representing 98% of patients with IVC tumor thrombi in the NWTS-4 [2]. Intracaval tumor thrombi may be classified according to the upper extent of the tumor thrombus in relation to the hepatic veins (modified Skinner classification of renal cell carcinoma): level 1, infrahepatic thrombus; level 2, retrohepatic thrombus; and level 3, suprahepatic or atrial thrombus [5,6].…”

“…The management of tumors that extend into the inferior vena cava (IVC) is determined by multiple factors including [1] extent of tumor thrombus, [2] possibility of tumor embolus, [3] tumor histology, [4] responsiveness of the tumor to chemotherapy, and [5] tumor stage at presentation. Imaging studies can accurately delineate the extent of vascular invasion and often suggest the diagnosis of these tumors.…”

Nonresponsiveness to Chemotherapy: A Formidable Surgical Challenge in Wilms’ Tumor with Intracardiac Extension

“…The management of tumors that extend into the inferior vena cava is determined by extent of tumor thrombus, possibility of tumor embolus, histology type, responsiveness of the tumor to chemotherapy, and tumor stage at presentation [1,2]. However, neoadjuvant chemotherapy followed by surgery is advocated in tumor with intravascular extension [1,3].…”