Spontaneous bilateral haemothorax as presentation of primary pleural epithelioid haemangioendothelioma

Pleural epithelioid haemangioendothelioma (EHE) is a rare tumour that originates in the vascular endothelium with an intermediate degree of malignancy between haemangioma and angiosarcoma. Smoking and asbestos exposure are unproven risk factors and diagnosis is usually confirmed by thoracoscopy, since pleural fluid (PF) cytology is often not conclusive. Immunohistochemistry can also help to confirm the diagnosis. We report an 85-year-old patient with bilateral pleural EHE diagnosed by thoracoscopy, who debuted with a spontaneous bilateral haemothorax, the second described so far, and we conducted a thorough review of the literature to describe the clinical, radiological and prognostic features, as well as the PF, of this rare tumour.