肺动脉肉瘤一例并文献复习_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

杨宠玉 ¹ , 王亚丽 ¹ , 李志英 ¹ , 张秋娣 ¹ , 张大川 ² ,  邱慧 ¹

1. ;
2. ;

Corresponding author：

邱慧, Email: naturehui@126.com

Keywords：

肺动脉肉瘤; 肺栓塞

DOI：

10.7507/1671-6205.202202022

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目的探讨肺动脉肉瘤（pulmonary artery sarcoma，PAS）的临床表现、影像学表现、病理学特点及鉴别诊断，提高临床医生对 PAS 的诊治水平。方法分析1 例经病理活检确诊的 PAS 患者的临床资料并复习相关文献。结果患者男，72 岁，因“咳嗽、胸闷半年”于 2018 年 2 月 27 日首次入院。既往体健。查体无特殊。胸部 CT 示左肺下叶见多发结节状高密度影，左肺动脉主干及分支内见斑块状低密度充盈影。诊断肺栓塞，予低分子肝素抗凝等治疗后好转出院。2018 年 4 月患者症状加重，复查 CT 肺动脉造影示左肺门增大，左肺动脉扩张，左肺动脉干管腔内巨大充盈缺损，肺动脉分叉处、左肺动脉多个分支及左心房亦可见多发充盈缺损，与首次入院时病灶相比明显进展。正电子发射断层显像检查示左肺动脉主干管腔扩张伴腔内低密度影，氟代脱氧葡萄糖代谢减低，左肺下叶结节样放射性浓聚影，SUVmax 10.3。行经肺动脉介入抽吸取栓术，病理确诊为 PAS。以“pulmonary artery sarcoma”“肺动脉肉瘤”“PAS”“pulmonary artery intimal sarcoma”“肺动脉内膜肉瘤”“PAIS”“pulmonary thromboembolism”“肺栓塞”“PTE”为关键词对国内外文献进行检索，共检索到相关文献 10 篇，涉及 506 例患者，其中男 265 例，女 241 例。PAS 患者的主要症状为咳嗽和胸闷。CT 肺动脉造影见肺动脉主干充盈缺损，可累及左右肺动脉及其分支。PAS 大都经手术确诊。病理见黏液背景中大量类似成纤维细胞或肌成纤维细胞的梭形细胞肉瘤，核异型性及有丝分裂像明显。免疫组织化学检测无特异性标志物，波形蛋白强阳性，CD31、CD34、Fli-1 不同程度阳性。手术切除是其主要的治疗手段，预后差。结论 PAS 是罕见的肺血管系统的恶性肿瘤。患者的临床表现、影像学检查和病理免疫组织化学检测无明显特异性，常被误诊为 PTE。及时诊断和早期治疗是延长患者生存时间的关键。

Citation： 杨宠玉, 王亚丽, 李志英, 张秋娣, 张大川, 邱慧. 肺动脉肉瘤一例并文献复习. Chinese Journal of Respiratory and Critical Care Medicine, 2022, 21(5): 347-352. doi: 10.7507/1671-6205.202202022 Copy

1.	Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol, 2015, 10(9): 1243-1260.
2.	Qin J, Ng CS, He P, et al. Pulmonary artery intimal sarcoma - a primeval or rediscovered tumor? A report of 14 new cases with literature review. Pathol Res Pract, 2021, 224: 153548.
3.	Han YX, Zhen YN, Liu XP, et al. Surgical treatment of primary pulmonary artery sarcoma. Gen Thorac Cardiovasc Surg, 2021, 69(4): 638-645.
4.	宋武, 刘盛, 邓隆, 等. 17 例肺动脉肉瘤的外科治疗. 中国循环杂志, 2019, 34(10): 999-1004.
5.	Deng L, Zhu JD, Xu JP, et al. Clinical presentation and surgical treatment of primary pulmonary artery sarcoma. Interact Cardiovasc Thorac Surg, 2018, 26(2): 243-247.
6.	Yin KH, Zhang ZQ, Luo RK, et al. Clinical features and surgical outcomes of pulmonary artery sarcoma. J Thorac Cardiovasc Surg, 2018, 155(3): 1109-1115.e1.
7.	周锦桃, 叶伶. 12 例肺动脉肉瘤患者的临床分析. 临床肿瘤学杂志, 2018, 23(2): 168-170.
8.	Secondino S, Grazioli V, Valentino F, et al. Multimodal approach of pulmonary artery intimal sarcoma: a single-institution experience. Sarcoma, 2017, 2017: 7941432.
9.	Srivali N, Yi ES, Ryu JH. Pulmonary artery sarcoma mimicking pulmonary embolism: a case series. QJM, 2017, 110(5): 283-286.
10.	Lee Y, Kim HJ, Yoon H, et al. Clinical characteristics and treatment outcomes of primary pulmonary artery sarcoma in Korea. J Korean Med Sci, 2016, 31(11): 1755-1760.
11.	Bandyopadhyay D, Panchabhai TS, Bajaj NS, et al. Primary pulmonary artery sarcoma: a close associate of pulmonary embolism—20-year observational analysis. J Thorac Dis, 2016, 8(9): 2592-2601.
12.	Mandelstamm M. Über primäre Neubildungen des Herzens. Virchows Arch Path Anat, 1923, 245: 43-54.
13.	Dewaele B, Floris G, Finalet-Ferreiro J, et al. Coactivated platelet-derived growth factor receptor α and epidermal growth factor receptor are potential therapeutic targets in intimal sarcoma. Cancer Res, 2010, 70(18): 7304-7314.
14.	Dembla V, Somaiah N, Barata P, et al. Prevalence of MDM2 amplification and coalterations in 523 advanced cancer patients in the MD Anderson phase 1 clinic. Oncotarget, 2018, 9(69): 33232-33243.
15.	Sai S, Imamura Y, Kiyota N, et al. Relationship between PDGFR expression and the response to pazopanib in intimal sarcoma of the pulmonary artery: a case report. Mol Clin Oncol, 2021, 14(1): 6.
16.	Penel N, Taieb S, Ceugnart L, et al. Report of eight recent cases of locally advanced primary pulmonary artery sarcomas: failure of Doxorubicin-based chemotherapy. J Thorac Oncol, 2008, 3(8): 907-911.
17.	Kim C, Kim MY, Kang JW, et al. Pulmonary artery intimal sarcoma versus pulmonary artery thromboembolism: CT and clinical findings. Korean J Radiol, 2018, 19(4): 792-802.
18.	Blackmon SH, Rice DC, Correa AM, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg, 2009, 87(3): 977-984.
19.	梁颖, 米玉红, 高元明, 等. 肺动脉肉瘤 8 例临床分析. 中国呼吸与危重监护杂志, 2013, 12(3): 284-288.
20.	Al-Mehisen R, Al-Halees Z, Alnemri K, et al. Primary pulmonary artery sarcoma: a rare and overlooked differential diagnosis of pulmonary embolism. Clues to diagnosis. Int J Surg Case Rep, 2019, 65: 15-19.
21.	姜维, 杨媛华, 李一丹, 等. 肺动脉肉瘤的超声心动图表现并文献回顾. 中国超声医学杂志, 2019, 35(11): 1048-1051.
22.	陈兢兢, 钟志成, 李静. 原发性肺动脉肉瘤的诊疗现状. 中华结核和呼吸杂志, 2016, 39(10): 818-821.
23.	Liu M, Luo CH, Wang Y, et al. Multiparametric MRI in differentiating pulmonary artery sarcoma and pulmonary thromboembolism: a preliminary experience. Diagn Interv Radiol, 2017, 23(1): 15-21.
24.	Attinà D, Niro F, Tchouanté P, et al. Pulmonary artery intimal sarcoma. Problems in the differential diagnosis. Radiol Med, 2013, 118(8): 1259-1168.
25.	甘辉立, 张健群, 黄小勇, 等. 肺动脉CT造影检查对肺动脉肉瘤鉴别诊断的意义. 心肺血管病杂志, 2015, 34(6): 480-485.
26.	Xi XY, Gao W, Gong JN, et al. Value of ¹⁸F-FDG PET/CT in differentiating malignancy of pulmonary artery from pulmonary thromboembolism: a cohort study and literature review. Int J Cardiovasc Imaging, 2019, 35(7): 1395-1403.
27.	刘阳祥, 王进, 吴晓虹, 等. 肺动脉肉瘤 4 例临床分析并文献复习. 国际呼吸杂志, 2018, 38(1): 10-14.
28.	Fukai R, Rokkaku K, Irie Y et al. Pulmonary artery sarcoma successfully treated by right pneumonectomy after definitive diagnosis. World J Clin Oncol, 2013, 4(4): 102-105.

1. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol, 2015, 10(9): 1243-1260.
2. Qin J, Ng CS, He P, et al. Pulmonary artery intimal sarcoma - a primeval or rediscovered tumor? A report of 14 new cases with literature review. Pathol Res Pract, 2021, 224: 153548.
3. Han YX, Zhen YN, Liu XP, et al. Surgical treatment of primary pulmonary artery sarcoma. Gen Thorac Cardiovasc Surg, 2021, 69(4): 638-645.
4. 宋武, 刘盛, 邓隆, 等. 17 例肺动脉肉瘤的外科治疗. 中国循环杂志, 2019, 34(10): 999-1004.
5. Deng L, Zhu JD, Xu JP, et al. Clinical presentation and surgical treatment of primary pulmonary artery sarcoma. Interact Cardiovasc Thorac Surg, 2018, 26(2): 243-247.
6. Yin KH, Zhang ZQ, Luo RK, et al. Clinical features and surgical outcomes of pulmonary artery sarcoma. J Thorac Cardiovasc Surg, 2018, 155(3): 1109-1115.e1.
7. 周锦桃, 叶伶. 12 例肺动脉肉瘤患者的临床分析. 临床肿瘤学杂志, 2018, 23(2): 168-170.
8. Secondino S, Grazioli V, Valentino F, et al. Multimodal approach of pulmonary artery intimal sarcoma: a single-institution experience. Sarcoma, 2017, 2017: 7941432.
9. Srivali N, Yi ES, Ryu JH. Pulmonary artery sarcoma mimicking pulmonary embolism: a case series. QJM, 2017, 110(5): 283-286.
10. Lee Y, Kim HJ, Yoon H, et al. Clinical characteristics and treatment outcomes of primary pulmonary artery sarcoma in Korea. J Korean Med Sci, 2016, 31(11): 1755-1760.
11. Bandyopadhyay D, Panchabhai TS, Bajaj NS, et al. Primary pulmonary artery sarcoma: a close associate of pulmonary embolism—20-year observational analysis. J Thorac Dis, 2016, 8(9): 2592-2601.
12. Mandelstamm M. Über primäre Neubildungen des Herzens. Virchows Arch Path Anat, 1923, 245: 43-54.
13. Dewaele B, Floris G, Finalet-Ferreiro J, et al. Coactivated platelet-derived growth factor receptor α and epidermal growth factor receptor are potential therapeutic targets in intimal sarcoma. Cancer Res, 2010, 70(18): 7304-7314.
14. Dembla V, Somaiah N, Barata P, et al. Prevalence of MDM2 amplification and coalterations in 523 advanced cancer patients in the MD Anderson phase 1 clinic. Oncotarget, 2018, 9(69): 33232-33243.
15. Sai S, Imamura Y, Kiyota N, et al. Relationship between PDGFR expression and the response to pazopanib in intimal sarcoma of the pulmonary artery: a case report. Mol Clin Oncol, 2021, 14(1): 6.
16. Penel N, Taieb S, Ceugnart L, et al. Report of eight recent cases of locally advanced primary pulmonary artery sarcomas: failure of Doxorubicin-based chemotherapy. J Thorac Oncol, 2008, 3(8): 907-911.
17. Kim C, Kim MY, Kang JW, et al. Pulmonary artery intimal sarcoma versus pulmonary artery thromboembolism: CT and clinical findings. Korean J Radiol, 2018, 19(4): 792-802.
18. Blackmon SH, Rice DC, Correa AM, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg, 2009, 87(3): 977-984.
19. 梁颖, 米玉红, 高元明, 等. 肺动脉肉瘤 8 例临床分析. 中国呼吸与危重监护杂志, 2013, 12(3): 284-288.
20. Al-Mehisen R, Al-Halees Z, Alnemri K, et al. Primary pulmonary artery sarcoma: a rare and overlooked differential diagnosis of pulmonary embolism. Clues to diagnosis. Int J Surg Case Rep, 2019, 65: 15-19.
21. 姜维, 杨媛华, 李一丹, 等. 肺动脉肉瘤的超声心动图表现并文献回顾. 中国超声医学杂志, 2019, 35(11): 1048-1051.
22. 陈兢兢, 钟志成, 李静. 原发性肺动脉肉瘤的诊疗现状. 中华结核和呼吸杂志, 2016, 39(10): 818-821.
23. Liu M, Luo CH, Wang Y, et al. Multiparametric MRI in differentiating pulmonary artery sarcoma and pulmonary thromboembolism: a preliminary experience. Diagn Interv Radiol, 2017, 23(1): 15-21.
24. Attinà D, Niro F, Tchouanté P, et al. Pulmonary artery intimal sarcoma. Problems in the differential diagnosis. Radiol Med, 2013, 118(8): 1259-1168.
25. 甘辉立, 张健群, 黄小勇, 等. 肺动脉CT造影检查对肺动脉肉瘤鉴别诊断的意义. 心肺血管病杂志, 2015, 34(6): 480-485.
26. Xi XY, Gao W, Gong JN, et al. Value of ¹⁸F-FDG PET/CT in differentiating malignancy of pulmonary artery from pulmonary thromboembolism: a cohort study and literature review. Int J Cardiovasc Imaging, 2019, 35(7): 1395-1403.
27. 刘阳祥, 王进, 吴晓虹, 等. 肺动脉肉瘤 4 例临床分析并文献复习. 国际呼吸杂志, 2018, 38(1): 10-14.
28. Fukai R, Rokkaku K, Irie Y et al. Pulmonary artery sarcoma successfully treated by right pneumonectomy after definitive diagnosis. World J Clin Oncol, 2013, 4(4): 102-105.

Chinese Journal of Respiratory and Critical Care Medicine

肺动脉肉瘤一例并文献复习

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