A rare pulmonary artery sarcoma, masquerading as pulmonary embolus.

We present a case of a 46 year old female with a presumed diagnosis of multiple unprovoked pulmonary emboli. She continued to deteriorate from a cardiorespiratory point of view despite optimal medical therapy. At surgical thrombectomy she was identified to have a intimal soft tissue lesion arising from the right pulmonary artery. This lesion was subsequently diagnosed as a pulmonary artery sarcoma. At nine months post surgery she has undergone six cycles of chemotherapy and has stable disease with no metastases. Pulmonary artery sarcoma (PAS) is a rare neoplasm which 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 heart strain. Median survival of these patients is 20 months with adjuvant chemo-radiotherapy. PMID: 31279785 [PubMed - as supplied by publisher]
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: Ann Thorac Surg Source Type: research