title: Systemic treatment options for epithelioid haemangioendothelioma: the Royal Marsden Hospital experience.
abstract: BACKGROUND
  Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour, which can arise at any site. There is little published data about the management of these tumours.
  
  
  METHODS
  A retrospective review of patients with histologically-proven EHE presenting to the Royal Marsden Hospital from January 1999 to January 2012.
  
  
  RESULTS
  Thirty-two patients (23 females) were identified with a median age of 44 (range=17-78). Twenty patients presented with diffuse disease. Median overall survival (OS) was 9.8 years in 10 patients with operable disease. Amongst those with inoperable disease (n=22), patients with liver disease had the longest median OS (9.8 years), while those with lung and mediastinal disease had the shortest OS (3.6 years). A variety of treatments were used for inoperable disease with infrequent radiological responses.
  
  
  CONCLUSIONS
  The clinical behaviour can vary depending on the site of disease. Surgery, if feasible, has the best outcome. In those with inoperable disease, a period of observation to assess the tumour behaviour is recommended. The role of medical therapy remains unclear.
authors.affiliation: Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London, U.K.
authors.forename: Nadia
authors.initials: N
authors.lastname: Yousaf
authors.affiliation: Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London, U.K.
authors.forename: Marco
authors.initials: M
authors.lastname: Maruzzo
authors.affiliation: Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London, U.K.
authors.forename: Ian
authors.initials: I
authors.lastname: Judson
authors.affiliation: Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London, U.K.
authors.forename: Omar
authors.initials: O
authors.lastname: Al-Muderis
authors.affiliation: Pathology Department, The Royal Marsden NHS Foundation Trust, London, U.K.
authors.forename: Cyril
authors.initials: C
authors.lastname: Fisher
authors.affiliation: Sarcoma Unit, The Royal Marsden NHS Foundation Trust, London, U.K. Charlotte.Benson@rmh.nhs.uk.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Benson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002173:Cancer Care Facilities; D018572:Disease-Free Survival; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000188:drug therapy* / Q000401:mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D008131:London; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: EHE; Epithelioid haemangioendothelioma; chemotherapy; observation; sarcoma; surgery; survival