Epithelioid haemangioendothelioma

Epithelioid haemangioendothelioma is a rare pulmonary neoplasm with less than 40 cases described world wide. We describe the only case to have presented with hypertrophic pulmonary osteoarthropathy who has been treated with azathioprine and has remained alive and well with no deterioration in pulmonary function since being diagnosed 16 years ago. The progression of the chest radiograph and spiral CT appearances of this rare neoplasm are described, and current views regarding the cellular origin of the neoplasm, its cytological appearance, clinical presentation and prognosis are discussed.