title: Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience.
abstract: BACKGROUND
  Undifferentiated embryonal sarcoma of the liver (UESL) represents less than 5% of all malignant hepatic tumors in childhood. It is considered an aggressive neoplasm with an unfavorable prognosis. The aim of this paper is to present a single center experience in the treatment of children with UESL.
  
  
  METHODS
  Ten children with UESL were treated between 1981 and 2012. Age at diagnosis ranged from 4 months to 17 years (median age, 6 years and 9 months). Surgery after neoadjuvant chemotherapy (CHT) was performed in 7 patients, and in 3 patients primary surgery was done. Adjuvant chemotherapy was administered in all 10 patients (CYVADIC, CAV, CAV/ETIF/IF+ADM, CDDP/PLADO). Right hemihepatectomy was performed in 1 patient, extended right hemihepatectomy in 6, and partial resection of the right lobe (segments V-VI, segment V) in 2 patients. One patient with unresectable tumor affecting both lobes was listed for liver transplantation (LTx).
  
  
  RESULTS
  Follow-up from diagnosis ranged from 50 to 222 months (mean 138 months). Among 9 patients treated with partial liver resection, distant metastases/local recurrence was not observed in any, and disease-free survival in this group is 100% (9 patients alive). The patient that underwent liver transplantation died of multiorgan failure 4 months postoperatively. However, this patient was misdiagnosed as having hepatoblastoma (HBL) and received PLADO chemotherapy. The overall survival rate is 90%.
  
  
  CONCLUSIONS
  Excellent results with long-term survival can be achieved in children with UESL with conventional therapy, including a combination of neoadjuvant and adjuvant chemotherapy and surgery, even in large extensively growing tumors.
authors.affiliation: Department of Pediatric Surgery and Organ Transplantation, Children's Memorial Health Institute, Warsaw, Poland. Electronic address: h.ismail@czd.pl.
authors.forename: Hor
authors.initials: H
authors.lastname: Ismail
authors.forename: Bożenna
authors.initials: B
authors.lastname: Dembowska-Bagińska
authors.forename: Dorota
authors.initials: D
authors.lastname: Broniszczak
authors.forename: Piotr
authors.initials: P
authors.lastname: Kaliciński
authors.forename: Przemysław
authors.initials: P
authors.lastname: Maruszewski
authors.forename: Przemysław
authors.initials: P
authors.lastname: Kluge
authors.forename: Ewa
authors.initials: E
authors.lastname: Swięszkowska
authors.forename: Andrzej
authors.initials: A
authors.lastname: Kościesza
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Lembas
authors.forename: Danuta
authors.initials: D
authors.lastname: Perek
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003951:Diagnostic Errors; D018572:Disease-Free Survival; D005260:Female; D006498:Hepatectomy / Q000379:methods; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D009373:Neoplasms, Germ Cell and Embryonal / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D012074:Remission Induction; D012189:Retrospective Studies; D012509:Sarcoma / Q000175:diagnosis / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome
keywords: Liver tumor; Sarcoma