ORIGINAL ARTICLE Embryonal sarcoma of the liver G. Chocarro • M. V. Amesty • F. Herna ´ndez • B. G. Goiboru Chenu • R. Ortı ´z • S. Herna ´ndez • A. Sa ´nchez • M. Ga ´mez • M. Lo ´pez Santamarı ´a • J. A. Tovar Accepted: 2 July 2013 Ó Springer-Verlag Berlin Heidelberg 2013 Abstract Purpose Embryonal sarcoma accounts for 6 % of liver tumors. This study reviews its features and the results of treatment in a referral center. Methods We retrospectively reviewed liver tumors trea- ted between 1995 and 2011. PRETEXT staging and biopsy were used to tailor chemotherapy according to SIOP pro- tocols. Radical surgery was performed when possible. Complications and cumulative survival were the outcome endpoints. Results Six out of 156 primary liver tumors (four males and two females) were sarcomas. The mean age at diag- nosis was 81 ± 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 (vin- cristine, 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 %. Conclusions Extended and metastatic embryonal sarcoma do poorly whereas localized tumors amenable to complete surgical removal after chemotherapy can cure. Keywords Hepatic tumor Á Embryonal sarcoma Á Radical surgery Á Chemotherapy Introduction Primary liver tumors in children are rare, representing 0.5–2 % of the neoplasms in them. Hepatoblastoma and hepatocellular carcinoma account approximately for 90 and 10 % of liver malignancies. The incidence of undifferen- tiated embryonal liver sarcoma (UELS) is very low, varying from 0.6 to 1.2 per million and accounting only for 9–15 % of malignant liver tumors in children depending on the series [1]. Recent advances in radiology, surgery, and chemother- apy permitted to improve survival of patients with malig- nant tumors of the liver. This is true for all categories including UELS. The purpose of the present study was to show the current results in the treatment of UELS based on the experience of a department with a large referral of liver patients. Materials and methods We performed a retrospective review of consecutive patients younger than 14 years with UELS treated in our hospital between 1995 and 2011. Gender, age at diagnosis, tumor location, clinical, biological, and radiological fea- tures were analyzed and the modalities of treatment and survival rates were recorded. G. Chocarro (&) Á M. V. Amesty Á F. Herna ´ndez Á B. G. G. Chenu Á R. Ortı ´z Á S. Herna ´ndez Á A. Sa ´nchez Á M. Ga ´mez Á M. L. Santamarı ´a Á J. A. Tovar Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain e-mail: glrchocarro@gmail.com 123 Pediatr Surg Int DOI 10.1007/s00383-013-3344-2