ซารโคมาของชนในหลอดเลอดแดงปอดทแสดงออกเปนกอนในชองกลางอกและลมเลอดเนองอกในหลอดเลอดแดงปอด: รายงานผปวยและการทบทวนวรรณกรรม

Background

Pulmonary artery intimal sarcoma (PAIS) is a rare malignant mesenchymal tumor originating from the intima of the pulmonary artery. It often mimics chronic thromboembolic disease and is associated with delayed diagnosis and poor prognosis. Case Presentation: We describe a 61-year-old man with a history of hypertension, dyslipidemia, and remote smoking who presented with progressive dyspnea, chest discomfort, orthopnea, and peripheral edema. Chest radiograph demonstrated mild cardiomegaly with rightward tracheal displacement. Contrast-enhanced computed tomography (CT) revealed a large heterogeneous enhancing mass involving the distal main pulmonary artery and proximal right pulmonary artery, associated with tumor thrombus and multiple pulmonary nodules. Surgical resection with right pulmonary artery reconstruction using bovine pericardium and left atrial appendage excision was performed. Histopathologic examination showed a high-grade spindle cell sarcoma. Immunohistochemistry revealed strong diffuse nuclear positivity for MDM2 and patchy SMA expression, with negativity for cytokeratin, CD31, CD34, desmin, and S100, supporting the diagnosis of PAIS.