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Primary Cardiac Angiosarcoma with Systemic Metastases; A Case Report and Review of the Literature

Noppadol Larbcharoensub MD*, Arunee Singhsnaeh BSc, MD*, Paisan Leelachaikul MD*

Affiliation : * Department of Pathology, Faculty of Medicine Ramathibodi Hospital, Mahidol University

Angiosarcoma is a relatively uncommon primary malignant tumor of the heart derived from the endothelial cells. The authors report a case of primary cardiac angiosarcoma of the right atrium with pulmonary, hepatic, cerebellar and bony metastases in a 46-year-old man who clinically presented as pulmonary hemorrhage. The patient’s occupational history had occasional exposure to polyvinyl chloride. The autopsy examination of the cardiac angiosarcoma characterized macroscopically by a right atrial tumor and histologically by anastomosing vascular channels and minute atypical pleomorphic endothelial cells. Immunohistological stainings were positive CD31, CD34 and focal positive factor VIII-related antigen. Clinical and pathologic features with briefly reviewed relevant literatures are discussed. This is the first reported description in the literature of a primary cardiac angiosarcoma with systemic metastases to multiple organs in Thailand.

Keywords : Angiosarcoma, Heart, Malignant, Metastases, Polyvinyl chloride


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JMed Assoc Thai
MEDICAL ASSOCIATION OF THAILAND
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