Combined in situ hypothermic liver preservation and cardioplegia for resection of hepatoblastoma with intra-atrial extension in a 3 year old child

• Intra-atrial tumoral extension of pediatric hepatic tumors has a poor prognosis.
• “En bloc” resection needs cardioplegia and normothermic extracorporeal circulation.
• Combination with in situ hypothermic liver preservation constitutes a safe method.
• Combination lowers risk of postoperative liver failure and reduces time pressure.

Cure of hepatoblastoma requires complete macro- and microscopic resection of the tumor, without tumor rupture. In case of hepatoblastoma with intra-atrial tumor extension (ITE), “en bloc” resection of the hepatic tumor and ITE, with minimal risk of postoperative liver failure, constitutes a surgical challenge. We report on a 3 year old child with hepatoblastoma of the right liver lobe, and ITE through the upper Inferior Vena Cava. Initial chemotherapy (SIOPEL IV HR) induced good response, but tumor persisted inside the right atrium with tight adhesions to the cardiac wall. “En bloc” right extended hepatectomy and removal of the ITE with reconstruction of the atrial and caval wall with autologous pericardial patch was performed under normothermic extracorporeal circulation and cardioplegia, combined with in situ hypothermic liver preservation of the remaining left liver. Complete tumor resection was achieved without tumor rupture. Postoperative liver function was immediately good and adjuvant chemotherapy was resumed per protocol. Eleven months after the end of treatment the child is in complete tumor remission. In children with hepatic tumor and ITE, combination of normothermic extracorporeal circulation with cardioplegia and in situ hypothermic liver preservation allows “en bloc” extended hepatectomy and removal of ITE, with limited risk of postoperative liver failure.