Epithelioid haemangioendothelioma.
10569 Background: Epithelioid haemangioendothelioma (EHE) is a rare vascular tumour which can arise in any site. There is little published data about the clinical behaviour and management of these tumours. Methods: A retrospective review of the management of patients with histologically proven EHE presenting to the Royal Marsden Hospital from January 1999 to January 2012. Demographics, site, treatment and survival outcomes were collected. Results: 28 patients (21 females) were identified with a mean (SD) age of 44 (17). 20 patients presented with diffuse disease involving one or more organs at presentation. The predominant site of disease was identified as liver n=8, mediastinum n=6, lung n= 2, limb n=1, pancreas n=1, spleen n=1, back n=1, abdominal wall n=1, oesophagus n=1, neck n=1, pelvis n=1, not known n=2. Median overall survival (Interquartile range) [OS (IQR)] in months was the longest in 10 patients who had localised operable disease at presentation, 116 (71). Amongst those with inoperable disease (n=16), patients with liver disease had the longest OS (IQR), 18 (43) months and those with lung and mediastinal disease had the shortest OS (IQR), 10 (2.5) and 11 (17) months respectively. A variety of treatment modalities were used for patients with inoperable disease and these are summarised in the Table. Conclusions: The clinical behaviour of EHE 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 clinical behaviour of the tumour is recommended. Non-steroidal anti-inflammatory drugs are a reasonable, relatively non-toxic first line treatment option. The use of anti-angiogenic drugs merits further exploration. [Table: see text]