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.