Undifferentiated embryonal sarcoma of the liver is a rare liver cancer in children and is most commonly diagnosed in those who are 5 to 10 years old. At diagnosis, the cancer has often spread throughout the liver and to the lungs. It’s very important to differentiate between embryonal sarcoma of the liver and biliary tract rhabdomyosarcoma, which are very similar clinically, but which require very different treatments.

Treatment of undifferentiated embryonal sarcoma of the liver involves complete surgical removal of the tumor, followed by chemotherapy (some combination of vincristine, cyclophosphamide, dactinomycin, doxorubicin, or ifosfamide is usually used). Tumors that cannot be completely removed after diagnosis are first shrunk with four cycles of chemotherapy and then reevaluated. Liver transplantation is sometimes necessary when tumors cannot be surgically removed.