Acute Pulmonary Artery Obstruction as the Primary Manifestation of a Rapidly Growing Intimal Sarcoma in a 54-Year Old Patient

A Case of Pulmonary Artery Sarcoma

“…In general, PAS is usually found in Middle-aged patients [6][7][8]. PAS is likely to be mistaken for chronic pulmonary thromboembolism because the clinical manifestations of PAS are remarkably similar to those of pulmonary thromboembolic disease [4,6,7,9,10].The histologic diagnosis can be determined by EBUS-TBNA and Endovascular Catheter Biopsy [11,12] .However in our case, the pathologic type of the biopsy was not consistent with the pathologic type after surgery.Surgical remains the mainstay of treatment for PAS since complete resection could offer the only chance for a potential cure. Surgical options range from radical resection, such as lobectomy and pneumonectomy, to endarterectomy [13].The median survival time has been reported between 1.5 and 12 months [14], and more recent cases have shown the bene t of improved management by aggressive surgical resection and adjuvant chemoradiotherapy with longer survival times reported [15].…”

“…PAS is very rare and highly malignant neoplasm with an aggressive behavior with an incidence of 0.001-0.03% among autopsy cases and making up roughly 15% of all primary cardiac tumors [3,4],.Less than 400 PAS cases have been reported since it was described by Mandelstamm in 1923.It generally derives from the endothelial cells of the pulmonary artery, and tend to spread within the pulmonary vasculature.Rarely, the pulmonary parenchyma itself is invaded (usually only in the later stages), and, unlike angiosarcomas elsewhere [5]. In general, PAS is usually found in Middle-aged patients [6][7][8]. PAS is likely to be mistaken for chronic pulmonary thromboembolism because the clinical manifestations of PAS are remarkably similar to those of pulmonary thromboembolic disease [4,6,7,9,10].The histologic diagnosis can be determined by EBUS-TBNA and Endovascular Catheter Biopsy [11,12] .However in our case, the pathologic type of the biopsy was not consistent with the pathologic type after surgery.Surgical remains the mainstay of treatment for PAS since complete resection could offer the only chance for a potential cure.…”