Clear cell sarcoma of the kidney with cavo-atrial tumor thrombus: complete resection in a child

Pediatric clear cell sarcoma of the kidney with cavoatrial thrombus

“…After having received some preresection chemotherapy, there is a possibility of the tumor thrombus being adhered to the IVC wall, and one may require excising the segment of IVC followed by the use of a graft to restore the continuity as has been observed in Wilms tumors. In the present case, as well as the one reported previously [5], fortunately, the thrombus could be removed using endarterectomy techniques, and the IVC and atrium could be repaired primarily without requiring a graft or patch interposition. Including the present case, 4 of 5 cases of CCSK with vascular thrombus had atrial extension [3][4][5].…”

“…Only 4 cases of CCSK with vascular thrombus have been reported in the English literature, and only 2 in the pediatric age group [5,6]. Of these 4 cases, 3 had tumor thrombus extending into the atrium [3][4][5]. Most intracaval and intraatrial thrombus can be delineated by CECT and a Doppler study.…”

“…Thrombus extending to right atrium is an uncommon finding in Wilms tumor with an incidence of 0.7% to 1.1% [2,6]. It is rare in CCSK, with only 3 such reported cases [3][4][5][6]. Herein we report a case of a 3-year-old boy with CCSK with vascular thrombus extending to the right atrium that was managed successfully by excision of the mass and thrombus under cardiopulmonary bypass.…”

“…Unlike Wilms tumor, vascular thrombus is almost unknown in CCSK. Only 4 cases of CCSK with vascular thrombus have been previously reported, and of these, only 2 were in the pediatric age group [3][4][5][6]. Thrombus extending to right atrium is an uncommon finding in Wilms tumor with an incidence of 0.7% to 1.1% [2,6].…”

Strategy for management of retroperitoneal tumors with caval tumor thrombus

“…In patient 4, histology revealed CCSK. 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].…”