Ann Hepatobiliary Pancreat Surg.  2021 Nov;25(4):528-531. 10.14701/ahbps.2021.25.4.528.

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

• Kazi M
• Kaushik P
• Saini S
• Premchandani D
• Qureshi SS

• KMID: 2523058
• DOI: http://doi.org/10.14701/ahbps.2021.25.4.528

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.

Keyword

Germ cell neoplasm; Hepatic veins; Tumor thrombosis; Inferior vena cava

Figure

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  Fig. 1 (A) Pre-chemotherapy computed tomography (CT) demonstrating a large right retroperitoneal mass (arrow) with ascites and liver metastasis (arrowhead). (B) CT showing thrombus filling the right atrium (arrow) with congested appearance of the liver and ascites. (C) Post-chemotherapy CT with persistent thrombosis of the hepatic veins (arrowheads) and a thrombus filling and expanding the retrohepatic inferior vena cava (arrow). (D) Reduction in size of the right atrial thrombus (arrow) and some resolution of congestion of the liver after chemotherapy.

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  Fig. 2 (A) Congested, dark liver intraoperatively prior to thrombectomy. (B) Raw surface of liver after right hepatectomy and retrohepatic inferior vena cava (IVC) thrombus being extracted with forceps. (C) Near complete resolution of the congestion with pink liver after completion of thrombectomy. (D) At the end of operation, right atrium being closed primarily, IVC at the level of hepatic cloaca being patched with pericardium, and retrohepatic IVC being removed without reconstruction.

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  Fig. 3 (A) Right hepatectomy specimen with attached tumor at the inferior surface (arrow). (B) Specimen of the tumor thrombus in the retrohepatic IVC, hepatic veins, and right atrium with adherent endocardium. LHV, left hepatic vein; MHV, middle hepatic vein; RHV, right hepatic vein; IVC, inferior vena cava.

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