Primary sarcoma of the pulmonary artery

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Primary sarcoma of the pulmonary artery is an extremely rare disorder, Usually not considered in the clinical differential diagnosis of cardiopulmonary disease. The manifestations of this malignant disease can mimic the more common conditions that include pulmonary embolism, primary pulmonary hypertension, metastatic pulmonary neoplasms, cardiomyopathy, constrictive pericarditis, and infective endocarditis. The sarcoma can be treated with early operative resection if the correct diagnosis is suspected clinically and confirmed angiographically.

Case report

A 66-year-old white man with congestive heart failure was transferred to the Cleveland Clinic for evaluation of a pulmonary nodule. His main complaint was extreme weakness. Except for obesity, he was in good health until 6 months before admission when gradual dyspnea, generalized weakness, leg swelling, orthopnea, and anorexia developed; he had lost 18 kg (40 pounds).

At his local hospital, a posteroanterior chest roentgenogram showed a coin lesion in the right lower lobe (Fig. 1). A second lesion, possibly a granuloma, was seen on a lateral view (Fig. 2). Studies for metastatic lung disease, including upper gastrointestinal examination, barium enema, intravenous urogram, liver-spleen scan, bone scan, and computed tomography (CT) of the abdomen were normal. A lung scan, however, showed no perfusion to the left lung and a defect in the right lung in what appeared to be the anterior segment of the right upper lobe.

On physical examination, his vital signs were normal. He appeared ashen, fatigued, and subdued. Two spider angiomas were visible on his face. Mild jugular venous distension was present when he was in . . .



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