Thorough study is required to decide the appropriate management of hepatic tumors in children. We present a case report of a hepatic embryonal undifferentiated sarcoma with unfavorable prognosis in a nine-year-old girl. After undergoing a detailed cancer characteristics and extension study, a two-stage surgery approach was decided. The hepatic tumor resection was the first procedure to be performed. One week later, under cardiopulmonary bypass, deep hypothermia, and circulatory arrest, thrombectomy of the inferior vena cava and right atrium was accomplished, plus thromboendarterectomy of the right pulmonary artery. During a four-year follow-up, the patient continues to be disease-free.
Keywords: cardiac tumors; cardiopulmonary bypass; endarterectomy; liver tumor.