Intimal pulmonary artery sarcoma presenting as dyspnea: case report

Abstract

Background: We report a case of pulmonary sarcoma which is a rare cause of the common symptom of dyspnea.

Case presentation: A fifty-one year old previously healthy male presented to the emergency room with complaints of dyspnea on exertion. A cardiac workup including an exercise stress test was negative but an echocardiography showed pulmonary stenosis. Cardiac MRI showed a large mass extending from the pulmonic valve to both the right and left pulmonary arteries suggestive of sarcoma. A complete resection and repair of the pulmonary artery was done and adjuvant chemotherapy with doxorubicin and ifosfamide was recommended. The patient is currently disease free after eighteen months.

Conclusion: Pulmonary artery sarcomas are a difficult diagnosis. The diagnosis may remain elusive for some time until the proper imaging techniques are utilized to make a diagnosis. Earlier and accurate diagnosis may lead to earlier interventions and improve survival.

Figure 1

Cardiac MRI showing the sarcoma arising from right ventricle outflow tract to both the right and left pulmonary artery.

Figure 2

CT scan showing extensive involvement of the main pulmonary artery and into the right trunk.

Figure 3

Gross pathology of the sarcoma invading the pulmonary artery.

Figure 4

Low field and high field microscopic findings showing spindle cells with hyperchromatic nuclei in myxoid background.