Systemic Treatment Options for Epithelioid Haemangioendothelioma: The Royal Marsden Hospital Experience

作者：Yousaf Nadia; Maruzzo Marco; Judson Ian; Al Muderis Omar; Fisher Cyril; Benson Charlotte^*

来源：Anticancer Research, 2015, 35(1): 473-480.

• Epithelioid haemangioendothelioma
• EHE
• survival
• observation
• chemotherapy
• surgery
• sarcoma

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.

• 出版日期2015-1