Primary left atrial haemangioendothelioma.

We describe the case of a 21-year-old female patient with epithelioid haemangioendothelioma of the roof of the left atrium. The patient underwent radical resection of the tumour with large disease-free margins, which required reconstruction of left and right atrial superior walls. The prognosis is unpredictable, but life expectancies ranging from 1 to 20 years have been described. There is no single treatment that can be prescribed but, because of their biologic behaviour, epithelioid haemangioendotheliomas must be regarded as fully malignant neoplasms and ought to be resected radically to prevent metastatic disease, and to improve life expectancy and quality of life.