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. PATIENTS AND 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. CONCLUSION: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.

journal_name

Anticancer Res

journal_title

Anticancer research

authors

Yousaf N,Maruzzo M,Judson I,Al-Muderis O,Fisher C,Benson C

subject

Has Abstract

pub_date

2015-01-01 00:00:00

pages

473-80

issue

1

eissn

0250-7005

issn

1791-7530

pii

35/1/473

journal_volume

35

pub_type

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