title: Treatment sequencing strategy for hepatic epithelioid haemangioendothelioma.
abstract: BACKGROUND
  The biology of hepatic epithelial haemangioendothelioma (HEHE) is variable, lying intermediate to haemangioma and angiosarcoma. Treatments vary owing to the rarity of the disease and frequent misdiagnosis.
  
  
  METHODS
  Between 1989 and 2013, patients retrospectively identified with HEHE from a single academic cancer centre were analysed to evaluate clinicopathological factors and initial treatment regimens associated with survival.
  
  
  RESULTS
  Fifty patients with confirmed HEHE had a median follow-up of 51 months (range 1-322). There was no difference in 5-year survival between patients presenting with unilateral compared with bilateral hepatic disease (51.4% versus 80.7%, respectively; P = 0.1), localized compared with metastatic disease (69% versus 78.3%, respectively; P = 0.7) or an initial treatment regimen of Surgery, Chemotherapy/Embolization or Observation alone (83.3% versus 71.3% versus 72.4%, respectively; P = 0.9). However, 5-year survival for patients treated with chemotherapy at any point during their disease course was decreased compared with those who did not receive any chemotherapy (43.6% versus 82.9%, respectively; P = 0.02) and was predictive of a decreased overall survival on univariate analysis [HR 3.1 (CI 0.9-10.7), P = 0.02].
  
  
  CONCLUSIONS
  HEHE frequently follows an indolent course, suggesting that immediate treatment may not be the optimal strategy. Initial observation to assess disease behaviour may better stratify treatment options, reserving surgery for those who remain resectable/transplantable. Prospective cooperative trials or registries may confirm this strategy.
authors.affiliation: Department of Surgery, NF/SG VA Medical Center, Gainesville, FL, USA; Department of Surgery, University of Florida College of Medicine, Gainesville, FL, USA.
authors.forename: Ryan M
authors.initials: RM
authors.lastname: Thomas
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Aloia
authors.forename: Mark J
authors.initials: MJ
authors.lastname: Truty
authors.forename: Warren H
authors.initials: WH
authors.lastname: Tseng
authors.forename: Eugene A
authors.initials: EA
authors.lastname: Choi
authors.forename: Steven A
authors.initials: SA
authors.lastname: Curley
authors.forename: Jean N
authors.initials: JN
authors.lastname: Vauthey
authors.forename: Eddie K
authors.initials: EK
authors.lastname: Abdalla
mesh_terms: D000046:Academic Medical Centers; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D004621:Embolization, Therapeutic; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000401:mortality / Q000556:secondary / Q000628:therapy*; D006498:Hepatectomy; D006801:Humans; D008113:Liver Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D018579:Patient Selection; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D013781:Texas; D013997:Time Factors; D016896:Treatment Outcome; D057832:Watchful Waiting; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents