• Hui Xu, Wenyu Yang, and Chenyun Wu.
• Department of Respiratory Medicine, the Second Affiliated Hospital of Wenzhou Medical University, Wenzhou, China. E-mail. xh_tomy@163.com.
• Saudi Med J. 2020 May 1; 41 (5): 532-536.

AbstractWe present a case of primary pulmonary arterial sarcoma (PPAS) treated with endostatin (endostar) injection and radiotherapy  discuss the diagnosis, clinical manifestations, and pathology of PPAS. The patient complained of cough with sputum, fever, and chest pain with hemoptysis. Numerous nodules were observed in the computed tomography (CT) scan. The patient was diagnosed with pulmonary embolism (PE) by computed tomography pulmonary angiography (CTPA). The pathology and immunohistochemistry results indicated soft tissue sarcomas, indicative of angiosarcoma. The nodules shrunk after 5 courses of endostatin and one course of radiotherapy, as determined in the CT scan. Primary pulmonary arterial sarcoma is clinically rare with nonspecific symptoms. Hence, it can be easily misdiagnosed as PE, and biopsy must be performed for confirmation. Current treatment methods, including surgery, are limited. Therefore, administration of endostatin injection combined with other therapies may be an alternative treatment methods.

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