Imaging and Surgical Treatment of Primary Pulmonary Artery Sarcoma

Primary pulmonary artery sarcoma is a rare tumor originating from the pulmonary artery tree. Given the low incidence, few centers have reported on more than a handful of cases. Because of its rarity it is also commonly misdiagnosed as pulmonary embolism. Multi-modality diagnostic imaging and recognition of specific imaging characteristics along with a high index of suspicion is required to make the correct diagnosis and expedite treatment. The primary imaging modality for most cardiac tumors such as primary pulmonary artery sarcoma is now MRI. It provides superb spatial resolution as well as functional assessment of the heart and pulmonary circulation. CT imaging also is part of routing imaging and remains as the most pertinent imaging modality to evaluate the lung parenchyma and presence of metastatic disease. Here we review the pertinent imaging modalities and tissue characteristics that facilitate recognition of primary pulmonary artery sarcoma. We also provide a short overview of surgical resection and reconstruction, which is the mainstay therapy, for this rare tumor.