Pulmonary artery sarcoma (PAS) is a rare tumor of the cardiovascular system Proper diagnosis can be a great challenge because clinical and radiological features are commonly indistinguishable from acute or chronic pulmonary embolism (PE). Here we present a case of PAS mimicking pulmonary embolism.
A 52‐year‐old man complained progressive exertional dyspnea and exercise intolerance for 1 week. There were associated palpitations. There was no fever, cough, recent travel, or history of heart failure. His medical history was notable for diabetes, chronic kidney disease, and tobacco use (20 pack‐years). The blood pressure was 102/55 mm Hg, pulse 111/min, respirations 22/min, and oxygen saturation 94% on nasal cannula (4 L/min). There was slight jugular venous distension with mild edema of both legs. The lungs and the remainder of the physical exam were normal. Significant lab results included creatinine 2.1 mg/dL and beta natriuretic peptide 437 pg/mL. The ECG showed sinus tachycardia, and the chest radiograph showed a slight prominence of the left hilum and a small left pleural effusion. By Wells criteria, a PE was likely. A V/Q scan showed near‐complete absence of left lung perfusion with normal ventilation. He was hospitalized and anticoagulated in the usual fashion. Duplex ultrasonography of the legs was normal. Three weeks later the patient developed hemoptysis, and his dyspnea had not improved significantly. Transthoracic echocardiography showed a moderately dilated right ventricle, moderate pulmonic stenosis, and a pulmonary artery systolic pressure of 43 mm Hg. Cardiac MRI demonstrated a large mass extending from the pulmonic valve, through the main pulmonary artery, and extending 3 cm into the left pulmonary artery. He underwent left pulmonary endarterectomy with arterial reconstruction using bovine pericardium. Pathology proved the mass tc be a pulmonary artery sarcoma. The patient's postoperative convalescence was uneventful, and he is currently being treated with sunitinib.
Pulmonary artery sarcoma is rare, with approximately 150 reported cases since the index case in 1923. Clinical and radiological features are nonspecific and very closely mimic those of typical pulmonary thromboembolic disease. Accordingly, PAS is initially misdiagnosed and mistreated as PE approximately 50% of the time. The most common signs and symptoms are dyspnea (76%), chest pain (60%), cough (53%), and hemoptysis (29%). Unilateral absence of perfusion, as in this case, is an uncommon and dramatic finding on perfusion scanning. Only 25% percent of these cases are a result of pulmonary embolism, with the remainder resulting from a variety of neoplastic, inflammatory, infectious, and congenital causes. In such cases, strong consideration should be given to expanding the differential diagnosis and pursing advanced imaging.
S. Lutchmedial, none; R. Brevetta, none; J. Sweet, none.