64 PULMONARY ARTERY INTIMAL SARCOMA: A CASE REPORT

Abstract

Abstract We present the case of a 56-years old man, with a history of smoking and chronic hypertension. From September 2021, he began experiencing shortness of breath, with marked limitation of ordinary physical activity. He underwent a cardiologic evaluation: an echocardiogram was performed, finding no alterations in the wall motions, but founding an increased atrioventricular gradient in the right heart and an augmented systolic pulmonary arterial pressure. Also, the acceleration time in the pulmonary artery was increased. Patient's physician prescribed him laboratory exams that resulted within normal ranges, with no increased levels of D-dimer and Chest X-ray in which it resulted to be a nodule, in the lower lobe of the left lung, that was biopsied in our center. We evaluated the patient with CT scan, CT angiography, PET-scan and cardiac MRI and discovered that the increased pressure in the pulmonary artery was due to a non-better specified tumor, which was then completely resected. After one month, the final diagnosis came back: poorly differentiated pleomorphic sarcoma, with high mitotic index and large areas of necrosis, characterized mainly by spindle cells and cells with epithelioid morphology; furthermore, there was a formation of vascular spaces surrounded by pleomorphic cells and areas of lower density cells with myxoid stroma and hemangiopericytoma-like aspects. Considering several factors such as anamnesis, localization of the tumor, growth from the tunica intima of the pulmonary artery and immunophenotypic expression, the tumor was diagnosed as a pulmonary arterial intimal sarcoma.