title: Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre.
abstract: BACKGROUND
  Primary hepatic sarcoma is a rare tumour with a poor prognosis.
  
  
  METHODS
  From 1997 to 2002 eight patients had liver resection for primary sarcoma of the liver at our institution. The clinical characteristics, imaging findings, surgical procedures, adjuvant therapy and outcome were retrospectively reviewed. There were two patients each with angiosarcoma (AS), leiomyosarcoma (LMS), and undifferentiated embryonal sarcoma (UES), one patient with epithelioid hemangioendothelioma (EHE) and one patient with malignant peripheral nerve sheath sarcoma (PNSS).
  
  
  RESULTS
  The most common presenting symptoms were right upper quadrant pain and fever. Typical imaging findings were a heterogenous mass with poorly defined margins, pseudocapsule and aberrant vasculature. Preoperative diagnosis of a primary liver sarcoma was made in 7/8 cases, either by fine needle aspiration (n = 5) or angiography (n = 2). Five right hepatectomies and three trisegmentectomies were performed. An R (0) resection was possible in three cases. Two patients developed complications and there was one death. Adjuvant chemoradiotherapy was administered to 5/7 patients. Systemic chemotherapy led to tumour regression in both patients with UES which enabled a second hepatic resection.
  
  
  CONCLUSIONS
  The majority of patients with primary liver sarcoma present with right upper quadrant pain, fever and a liver mass. Differentiating the rare primary liver sarcoma from the much more common hepatocellular carcinoma (HCC) may aid in planning therapy. Patients with resectable tumours should be referred for surgery. Liver resection combined with adjuvant chemotherapy are the mainstays of treatment for UES in the adult.
authors.affiliation: Department of Surgery, Hadassah University Hospital, Jerusalem, Israel. galmogy@surgery.usc.edu
authors.forename: Gidon
authors.initials: G
authors.lastname: Almogy
authors.forename: Sivan
authors.initials: S
authors.lastname: Lieberman
authors.forename: Maya
authors.initials: M
authors.lastname: Gips
authors.forename: Orit
authors.initials: O
authors.lastname: Pappo
authors.forename: Yair
authors.initials: Y
authors.lastname: Edden
authors.forename: Oded
authors.initials: O
authors.lastname: Jurim
authors.forename: B
authors.initials: B
authors.lastname: Simon Slasky
authors.forename: B
authors.initials: B
authors.lastname: Uzieli
authors.forename: Ahmed
authors.initials: A
authors.lastname: Eid
mesh_terms: D000328:Adult; D000368:Aged; D017024:Chemotherapy, Adjuvant; D003937:Diagnosis, Differential; D005260:Female; D006498:Hepatectomy / Q000379:methods; D006801:Humans; D007902:Length of Stay; D008111:Liver Function Tests; D008113:Liver Neoplasms / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012509:Sarcoma / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome