PULMONARY ARTERY SARCOMA: A SINGLE-INSTITUTION EXPERIENCE OF A MULTIMODAL THERAPEUTIC APPROACH TO A RARE MALIGNANT TUMOR

Pulmonary artery sarcoma (PAS) is a rare and aggressive malignant tumor that arises from the intimal or subintimal layer of the pulmonary artery wall. In most cases PAS presents with pulmonary hypertension (PH), mimicking a thromoboembolic disease. The therapeutic approach is mainly based on surgery, either pneumonectomy or pulmonary endarterectomy (PEA). The prognosis reported in published series is very poor. Among 1027 patients referred to our Cardiac Surgery Unit for suspected chronic pulmonary hypertension potentially (PH) requiring PEA from January 2010 to January 2016, 574 had a confirmed PH, and 448 underwent TEAP. Twenty-two patients had a radiological diagnosis of suspected tumor growing in the pulmonary artery, by high-resolution CT scan of the thorax. Among 22 patients with radiological diagnosis of tumor, 20 were eligible for surgery. As shown in our study, PEA represents the safest and more effective surgical procedure. It should be preferred also because it consents a rapid multidisciplinary pathway of care, along with CT and RT. Further clinical research is mandatory to improve the outcome of patients and for a better understanding of the role of systemic anticancer therapy and RT.