Pulmonary Artery Intimal Sarcoma Presenting as a Mediastinal Mass with Pulmonary Artery Tumor Thrombus: A Case Report and Review of the Literature
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Abstract
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.
Conclusion: PAIS should be considered in patients presenting with pulmonary artery obstruction and mediastinal mass. MDM2 immunohistochemistry is a valuable diagnostic marker, and early surgical intervention remains the mainstay of treatment.
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- เนื้อหาและข้อมูลในบทความที่ลงพิมพ์กับวารสารโรคหัวใจและทรวงอกถือเป็นข้อคิดเห็น และความรับผิดชอบของผู้เขียนบทความโดยตรงซึ่งกองบรรณาธิการวารสารไม่จำเป็นต้องเห็นด้วย หรือร่วมรับผิดชอบใด ๆ
- บทความ ข้อมูล เนื้อหา รูปภาพ ฯลฯ ที่ได้รับการตีพิมพ์ในวารสารสถาบันโรคทรวงอกถือเป็นลิขสิทธิ์ของวารสารวิชาการ หากบุคคลหรือหน่วยงานใดต้องการนำทั้งหมดหรือส่วนหนึ่ง ส่วนใดไปเผยแพร่ กรุณาอ้างอิงบทความนั้น ๆ
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