Leiomyosarcoma of pulmonary truncus

Insidious and progressive right heart failure of obscure origin warrants differential diagnosis of primary sarcoma of the pulmonary artery in patients of both sexes and 20 years of age and older. We report a case of primary leiomyosarcoma of the pulmonary truncus in a 67 years old woman. She had been well in general until when she started having fluctuating and progressive right heart failure three years ago. Clinical data included systolic murmurs in the pulmonary valve area, right ventricular hypertrophy, diminished x‐ray density of the pulmonary vasculature, moderate elevation of serum LDH, alkaline phosphatase, and uric acid, and prolonged or unobtainable circulation time from the arm to lungs. The immediate cause of death was attributable to right heart failure due to pulmonary arterial stenosis caused by a locally invasive leiomyosarcoma apparently arising in the pulmonary truncus. Contributing to this right heart failure were 280 ml of serosanguinous pericardial effusion and fibrinous pericarditis. Acta pathol. jpn. 34: 863∼867, 1984.