Extensive cavo-atrial and hepatic venous tumor thrombus in a mismanaged retroperitoneal pediatric germ cell tumor: A unique surgical challenge

Abstract

Retroperitoneal germ cell tumor with tumor thrombosis of the inferior vena cava (IVC) represents a rare phenomenon. Its extension to the hepatic veins (HVs) has not been reported yet. In the present case, a 30-month-old girl had a recurrent retroperitoneal yolk sac tumor with liver metastasis. In addition, there were tumor thrombi in the IVC, right atrium (RA), and all three HVs. The child was operated after a satisfactory response to chemotherapy. Excision of the retroperitoneal tumor with right hepatectomy, retrohepatic caval resection, HV, and RA thrombectomy was performed under a cardiac bypass. HV cloaca was patched with pericardium while the IVC resection was tolerated without reconstruction. Her postoperative recovery was normal. The child currently remains disease-free and symptom-free at 12 months. This case demonstrates the technique of hepatic venous thrombectomy and the feasibility of radical surgery even for a metastatic disease when tumor biology is favorable. Despite wide-spread tumor thrombosis that would have been deemed unresectable, complete resections are possible in referral centers.

Retroperitoneal yolk sac tumour encroaching the liver and adrenal gland with tumour thrombus in cavo-atrial region and hepatic veins

“…Paediatric retroperitoneal germ cell tumours with tumour thrombus extending to the atrio-caval or hepatic veins are rare. Only one paediatric case is reported in the literature till now 3. Our patient had locally advanced disease, and tumour was extended to the liver and adrenal gland along with thrombus in IVC and HVs.…”

“…As per Sudour-Bonnange et al , the current strategy is the initial platinum-based chemotherapy followed by gross total resection of all macroscopic residua10; we also followed the same, and tumour reduced in size, along with IVC thrombus downgraded to level 2 from level 3 after neoadjuvant chemotherapy. So, we did not need cardiac bypass, while Kazi et al needed cardiac bypass in their patient due to level 3 IVC thrombus 3. Chronic obstruction of the IVC often leads to the development of collaterals by the time the tumour is resected.…”