Superior vena caval approach for resecting intracardially extended Ewing sarcoma

Intracardiac extension of Ewing sarcoma is extremely rare. Herein, we report the case of a pediatric patient with mediastinal Ewing sarcoma which extended to right atrium via the azygos vein. Surgical resection was performed through longitudinal incision on anterior surface of the superior vena cava under cardiopulmonary bypass. Resection was feasible because the tumor was sufficiently elastic and non-adherent to the inner surface of the heart. The patient was received chemotherapy and proton beam radiation postoperatively and is doing well with no tumor recurrence for 5 years after surgery.