A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma
Tao Luan,
Jianqing Hao,
Yingying Gu,
Ping He,
Yijia Li,
Liqiang Wang,
Haiyi Deng,
Wenhui Guan,
Xinqing Lin,
Xiaohong Xie,
Yu Deng,
Shuaiying Wang,
Changchun Wang,
Jingxu Li,
Ru Li,
Yang Luan,
Gang Yang,
Yunhui Zhang,
Nanshan Zhong,
Chengzhi Zhou
Affiliations
Tao Luan
Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology
Jianqing Hao
Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology
Yingying Gu
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Ping He
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Yijia Li
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Liqiang Wang
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Haiyi Deng
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Wenhui Guan
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Xinqing Lin
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Xiaohong Xie
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Yu Deng
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Shuaiying Wang
Anesthesia Surgery Center, Zhejiang Cancer Hospital
Changchun Wang
Anesthesia Surgery Center, Zhejiang Cancer Hospital
Jingxu Li
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Ru Li
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Yang Luan
Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology
Gang Yang
Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology
Yunhui Zhang
Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology
Nanshan Zhong
Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and Technology
Chengzhi Zhou
National Center for Respiratory Medicine, National Clinical Research Center for Respiratory Disease, State Key Laboratory of Respiratory Disease, Guangzhou Institute of Respiratory Health, The First Affiliated Hospital of Guangzhou Medical University
Abstract Objective The aim of this study is to explore the clinicopathological features, radiographic manifestations, treatment options, and prognosis of primary pulmonary angiosarcoma (PPAS). Method We summarized and analyzed the clinical data of 11 patients with primary pulmonary angiosarcoma treated at the First Affiliated Hospital of Guangzhou Medical University between January 2018 and January 2024. A retrospective analysis was conducted in conjunction with a review of the relevant literature. Results Among the 11 cases, the primary symptoms were nonspecific respiratory issues, with chest pain, cough, and shortness of breath being the most common. Initial diagnosis was correct in only 2 cases, while the remaining 9 cases were misdiagnosed as other conditions, such as lung nodules, infections, or pulmonary embolism. All patients were ultimately diagnosed via pathology, with CD31 and CD34 showing a high positivity rate of 60%. Of the 11 patients, 9 have died, and 2 remain alive. The average survival period was 13.2 months.Based on the number of lesions, PPAS was categorized into three types: solitary, multiple, and invasive pulmonary artery. The average survival period for the solitary type (2 cases) was 15.5 months, for the multiple type (5 cases) was 6.6 months, and for the pulmonary artery type (4 cases) was 20.5 months. Among the 3 patients who underwent surgery, the median overall survival (OS) was 23.0 months, whereas for the 8 non-surgical patients, the median OS was 9.7 months. Conclusion Primary pulmonary angiosarcoma is a rare, highly aggressive vascular malignancy characterized by nonspecific clinical symptoms and a tendency for metastasis and pulmonary artery embolism. Currently, no effective treatment exists, and the overall prognosis is poor.
