대한흉부영상의학회 Korean Society of Thoracic Radiology

Discussion

Diagnosis With Brief Discussion

Diagnosis

Intimal Sarcoma of Pulmonary Artery

Radiologic Findings

On PA Chest radiograph, the cardiac silhouette is grossly enlarged with the shape of water bottle, which suggests pericardial effusion. The lungs are quite clear. Contrast enhanced CT scans demonstrate polypoid mass in the main pulmonary artery and it extends into the right and left pulmonary artery along the pulmonary arterial wall. Dilatation of right atrium and ventricle is most likely due to obstruction of pulmonary circulation by the mass. Note also pericardial effusion.
Photomicrograph of a specimen of the tumor shows a multiple polypoid mass within the pulmonary artery.

Brief Review

Primary malignant tumors of the pulmonary arteries are almost exclusively sarcomas. Pulmonary artery sarcoma affects patients aged 45-55 years with a female to male ratio 2:1. Dyspnea, chest pain, and cough are the most common symptoms at presentation, with pulmonary embolism being the usual initial clinical concern.
Pulmonary artery sarcomas arise from the intimal or subintimal areas of the right, left, or main pulmonary arteries, and growth frequently extends distally into the small pulmonary arteries. Direct tumor extension into the lung parenchyma has been reported to be as high as 56%; however, the tumor can attain significant intravascular size without extraluminal extension despite its highly malignant nature. The histologic diagnosis is usually intimal sarcoma, angiosarcom, or leiomyosarcoma. CT demonstrates an intraluminal filling defect within the large pulmonary arterial branches. The finding of contrast enhancement in an intraluminal mass on MR images indicates a neoplasm.

References

1. Baker PB, Goodwin RA. Pulmonary artery sarcomas. A review and report of a case. Arch Pathol Lab Med 1985; 109:35-39
2. Shmooker BM, Marsh HB, Roberts WC. Primary sarcoma of the pulmonary trunk and/or right or left main pulmonary artery - a rare cause of obstruction to right ventricular outflow. Report on two patients and analysis of 35 previously described patients. Am J Med 1977; 63:263-272
3. Burke AP, Virmani R. Sarcomas of the great vessels: a clinicopathologic study. Cancer 1993; 71:1761-1773

Keywords

Vascular, Malignant tumor,