Embryonal sarcoma accounts for 6 % of liver tumors. This study reviews its features and the results of treatment in a referral center. We retrospectively reviewed liver tumors treated between 1995 and 2011. PRETEXT staging and biopsy were used to tailor chemotherapy according to SIOP protocols. Radical surgery was performed when possible. Complications and cumulative survival were the outcome endpoints. Six out of 156 primary liver tumors (four males and two females) were sarcomas. The mean age at diagnosis was 81 +/- A 8.5 months. The most frequent finding was abdominal mass. Alfa-fetoprotein was normal. Imaging depicted heterogeneous tumors with septa, necrosis, and hemorrhagic areas. The diagnosis was ascertained by biopsy. Three tumors were located in the right lobe (PRETEXT II): two were bilobar (PRETEXT III) and one was in the left lobe (PRETEXT I). Two children had metastases at diagnosis and high-risk chemotherapy (vincristine, carboplatin, epirubicin) was administered with poor response. They died without operation 4 and 10 months later. Four patients with local disease underwent typical liver resections after chemotherapy (iphosphamide, vincristine, actinomycin D, and doxorrubicin). Overall actuarial survival at 70 months was 66.6 %. Extended and metastatic embryonal sarcoma do poorly whereas localized tumors amenable to complete surgical removal after chemotherapy can cure.
