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-  2019 

Primary pulmonary angiosarcoma: case reports and review of the literature

DOI: 10.1177/0218492319836910

Keywords: Diagnosis,differential,hemangiosarcoma,immunohistochemistry,mediastinal neoplasms,pulmonary artery,vascular neoplasms

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Abstract:

Pulmonary artery angiosarcoma is a rare malignant vascular tumor with an aggressive clinical course and a poor prognosis. Hemoptysis and shortness of breath have been reported as common clinical presentations. The exact clinicopathology is unknown. A tissue specimen obtained by percutaneous fine-needle aspiration cytology or open/thoracoscopic biopsy can confirm the diagnosis based on histopathological and immunohistochemical features. The differential diagnosis includes pulmonary thromboembolism, vascular malformations, and lung carcinoma. There is a paucity of literature describing this tumor, with only a few case reports available. There is also a lack of standardized guidelines for management, which further worsens the survival outcome. We report 3 cases of pulmonary artery angiosarcoma and review the recent literature

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