Aggressive form of pleural epithelioid haemangioendothelioma: complete response after chemotherapy

Abstract

Epithelioid haemangioendotheliomas are rare tumours of endothelial origin. They can develop in any tissue but occur principally in the lung and liver. Their usual course is a slow progression, so that they can be treated by surgery. In aggressive forms, no treatment has proved efficient to date. This study, describes a case of bilateral pleural epithelioid haemangioendothelioma that extended to the peritoneum. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. After six courses of carboplatine plus etoposide, a complete response was obtained. The complete remission is still lasting at 18 months after the diagnosis and the patient is healthy.