A Rare Pulmonary Artery Sarcoma Masquerading as Pulmonary Embolus

Abstract

We present the case of a 46-year-old woman with a presumed diagnosis of multiple unprovoked pulmonary emboli. Her cardiorespiratory function continued to deteriorate despite optimal medical therapy. At surgical thrombectomy she was identified to have an intimal soft tissue lesion arising from the right pulmonary artery. This lesion was subsequently diagnosed as a pulmonary artery sarcoma, a rare neoplasm that carries a poor prognosis. It typically presents at an advanced stage with pulmonary vascular obstruction. Surgical debulking is the mainstay of therapy to restore ventilation perfusion mismatching and relieve right-sided heart strain. Median survival of these patients is 20 months with adjuvant chemoradiotherapy. At 9 months after surgery, she has undergone 6 cycles of chemotherapy and has stable disease with no metastases.