SESSION TITLE: Thoracic Surgery Cases
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Tuesday, October 27, 2015 at 07:30 AM - 08:30 AM
INTRODUCTION: Pulmonary artery sarcomas are rare tumors with less than 300 cases reported in the literature. They arise from the pulmonary vasculature and can extend proximally and distally, rapidly resulting in right heart failure and death. Although most interventions are considered palliative, surgical excision can prolong survival. Here we present a case of pulmonary artery angiosarcoma with right ventricular failure that was treated with PTE.
CASE PRESENTATION: A 28 year-old Malaysian male was referred for evaluation of CTEPH and possible PTE. Until 8 months prior to presentation he had been very active, but at that time he started developing significant and progressive dyspnea on exertion which culminated to syncope. On initially work-up in he was thought to have a “saddle” PE with extension into the proximal branches of the ascending and descending PA based on CT pulmonary angiography. He received alteplase without improvement, and after two months on anticoagulation and persisting symptoms he underwent pulmonary embolectomy. Histopathology from the surgical specimen at the time demonstrated thrombus without neoplasia, but the clot was noted to be adherent to the vessel wall and difficult to remove. After some initial relief, his symptoms recurred and were compounded by anorexia and severe lower extremity edema. Follow up echocardiography showed elevated PA pressures and a mass within the the right ventricular outflow tract (RVOT). Right heart catheterization and PA angiogram showed mean PA pressure of 44mmHg, cardiac output of 2.4 and significant bilateral filling defects. He underwent bilateral PTE, RVOT mass resection and pulmonary valve replacement. His pathology was consistent with PA sarcoma, and despite developing acute renal, liver and respiratory failure, DIC and cardiogenic shock post-operatively, he improved and was eventually discharged home.
DISCUSSION: Pulmonary artery sarcoma is a rare and aggressive tumor, often misdiagnosed for pulmonary emboli and is usually confirmed on surgical and autopsy specimens. The disease is highly fatal even if treated, with studies demonstrating an average survival of 17 months after surgical resection and chemotherapy with or without radiation.
CONCLUSIONS: Although there are case reports of surgical cure this is rare, and surgical excision with PTE surgery or with pneumonectomy remains the best option for prolonging survival and providing some symptom relief.
Reference #1: Devendra G et al. Pulmonary artery sarcomas: the UCSD experience. Am J Respir Crit Care Med 2002;165:A24.
DISCLOSURE: The following authors have nothing to disclose: Alexandra Perry, Nick Kim, William Auger, David Poch, Gert Pretorius, Demosthenes Papmatheakis
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