A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma
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 trea...
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BMC
2024-12-01
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| Series: | BMC Cancer |
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| Online Access: | https://doi.org/10.1186/s12885-024-13277-4 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Tao Luan |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-1a4e825f5caf48138d10b954c998a09d |
| institution | Kabale University |
| issn | 1471-2407 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj-art-1a4e825f5caf48138d10b954c998a09d2025-01-05T12:33:12ZengBMCBMC Cancer1471-24072024-12-0124111010.1186/s12885-024-13277-4A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcomaTao Luan0Jianqing Hao1Yingying Gu2Ping He3Yijia Li4Liqiang Wang5Haiyi Deng6Wenhui Guan7Xinqing Lin8Xiaohong Xie9Yu Deng10Shuaiying Wang11Changchun Wang12Jingxu Li13Ru Li14Yang Luan15Gang Yang16Yunhui Zhang17Nanshan Zhong18Chengzhi Zhou19Faculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and TechnologyFaculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and TechnologyNational 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 UniversityNational 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 UniversityNational 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 UniversityNational 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 UniversityNational 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 UniversityNational 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 UniversityNational 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 UniversityNational 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 UniversityNational 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 UniversityAnesthesia Surgery Center, Zhejiang Cancer HospitalAnesthesia Surgery Center, Zhejiang Cancer HospitalNational 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 UniversityNational 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 UniversityFaculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and TechnologyFaculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and TechnologyFaculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and TechnologyFaculty of Life science and Technology, Kunming University of Science and Technology, The Affiliated Hospital of Kunming University of Science and TechnologyNational 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 UniversityAbstract 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.https://doi.org/10.1186/s12885-024-13277-4Rare tumorPulmonary vascular sarcomaSurvival periodImaging |
| spellingShingle | 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 A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma BMC Cancer Rare tumor Pulmonary vascular sarcoma Survival period Imaging |
| title | A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma |
| title_full | A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma |
| title_fullStr | A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma |
| title_full_unstemmed | A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma |
| title_short | A clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma |
| title_sort | clinical analysis and literature review of eleven cases with primary pulmonary angiosarcoma |
| topic | Rare tumor Pulmonary vascular sarcoma Survival period Imaging |
| url | https://doi.org/10.1186/s12885-024-13277-4 |
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