title: Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma.
abstract: BACKGROUND
  Hepatic epithelioid hemangioendothelioma (PHEHE) is a multifocal, low-grade malignant neoplasia characterized by its epithelial-like appearance and vascular endothelial histogenesis. The outcome of 16 patients treated with orthotopic liver transplantation (OLT) is the subject of this report.
  
  
  METHODS
  A retrospective study of 16 patients with HEHE (7 men, 9 women) with ages ranging from 24 to 58 years (mean 37 +/- 10.6 years). Follow-up intervals ranged from 1 to 15 years (median of 4.5 years).
  
  
  RESULTS
  Actual patient survival at 1, 3, and 5 years was 100, 87.5, and 71.3%, respectively. Disease-free survival at 1, 3, and 5 years was 81.3, 68.8, and 60.2%, respectively. The 90-day operative mortality was 0. Involvement of the hilar lymph nodes or vascular invasion did not affect survival. The 5-year survival of HEHE compares favorably with that of hepatocellular carcinoma at the same stage (stage 4A): 71.3 versus 9.8% (p = 0.001)
  
  
  CONCLUSIONS
  The long-term survival obtained in this series justifies OLT for these tumors even in the presence of limited extrahepatic disease.
authors.affiliation: Department of Surgery, University of Pittsburgh School of Medicine, Pennsylvania, USA.
authors.forename: J R
authors.initials: JR
authors.lastname: Madariaga
authors.forename: I R
authors.initials: IR
authors.lastname: Marino
authors.forename: D D
authors.initials: DD
authors.lastname: Karavias
authors.forename: M A
authors.initials: MA
authors.lastname: Nalesnik
authors.forename: H R
authors.initials: HR
authors.lastname: Doyle
authors.forename: S
authors.initials: S
authors.lastname: Iwatsuki
authors.forename: J J
authors.initials: JJ
authors.lastname: Fung
authors.forename: T E
authors.initials: TE
authors.lastname: Starzl
mesh_terms: D000328:Adult; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000401:mortality / Q000601:surgery*; D006801:Humans; D008113:Liver Neoplasms / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D015996:Survival Rate; D013997:Time Factors