Pulmonary artery sarcoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Pulmonary artery sarcomas are rare cancerous tumors in the pulmonary artery.

The first case that was published occurred in 1923 by M. Mandelstamm.^[1] Since this point, only about 250 cases have been reported.

Since the symptoms of a pulmonary artery sarcoma often mimic the symptoms of a pulmonary embolism, steps need to be taken to differentiate the diseases. Some of the clinical data that can help in differentiating the two from each other are as follows:^[1]

• Fever
• Elevated erythrocyte sedimentation rate
• Anemia
• Weight loss
• Absence of a procoagulant state
• Lack of history for deep vein thrombosis

The prognosis for a pulmonary artery sarcoma is very poor. The life expectancy is typically reported in weeks instead of months or years.^[1]

• Hemoptysis
• Dyspnea
• Chest pain
• Cough

Many different imaging studies can be used to evaluate a pulmonary artery sarcoma, but the standard test is the CT scan.

Typically in cases of cancer, chemotherapy is the preferred treatment method. In the case of a pulmonary artery sarcoma, treatment with chemotherapy is not ideal. There is not currently a preferred treatment regimen.^[1]

Many of the interventional treatments involve extensive surgical techniques. Some of these techniques include:

• Palliative stenting
• Pneumonectomy
• Debulking
• Endarterectomy
• Excision