Multi-disciplinary treatment of undifferentiated embryonal sarcoma of the liver with tumor embolus extending to the heart

Abstract Undifferentiated embryonal sarcoma of the liver (UESL) is a highly malignant neoplasm occurring predominantly in children. Complete resection is the only curative treatment. We encountered an 11-year-old boy with UESL arising in the right lobe of the liver extending to the pulmonary valve. He was treated with IREC (irinotecan, etoposide, and carboplatin) neoadjuvant chemotherapy, concurrent extended radical right hepatectomy, and intracardiac tumorectomy under cardiopulmonary bypass, and adjuvant IREC chemotherapy. He remains in remission without complications 48 months after diagnosis. Multidisciplinary treatment, particularly the combination of radical surgery and effective neoadjuvant chemotherapy, may contribute to UESL patients' survival.