Surgery in primary leiomyosarcoma of the heart

Primary leiomyosarcoma of the heart is uncommon and we were able to find only 15 cases reported in the literature. Few of these cases were discovered during life and, as a result, even fewer have been treated surgically. We report a case of a right atrial leiomyosarcoma which was proven pathologically (histologically, immunohistochemically and ultrastructurally) after surgical resection. The clinical presentation, morphological, appearances and outcome are compared with those reported in the literature.