Case of mediastinal epithelioid hemangioendothelioma associated with hypertrophic osteoarthropathy and literature review

Abstract

Hypertrophic osteoarthropathy is commonly associated with malignancies of the lungs and stomach. A few cases associated with epithelioid hemangioendothelioma, a rare vascular soft tissue neoplasm, have been reported. Presented here is the first report of a case of hypertrophic osteoarthropathy as the initial symptom of epithelioid hemangioendothelioma of the mediastinum. A 64-year-old woman presented with pain around the long bones and diffuse puffiness of bilateral hands and feet. She had a history of multiple malignancies, all of which were in remission. She had clubbing of her fingernails and toenails. Her chest X-ray was negative. A positron emission tomography (PET) scan performed showed metabolically active soft tissue that extensively infiltrated the mediastinum, with several sites of disease involving the pericardium. Mediastinal biopsy and subsequent pleural fluid cytology obtained by thoracocentesis was consistent with metastatic epithelioid hemangioendothelioma. Our case supports that the PET scan has a valuable role in localizing malignancies in such patients who present with hypertrophic osteoarthropathy and no lung or stomach involvement. © 2012 Tianjin Lung Cancer Institute and Wiley Publishing Asia Pty Ltd.