肺動脈肉瘤一例并文獻復習_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

楊寵玉 ¹ , 王亞麗 ¹ , 李志英 ¹ , 張秋娣 ¹ , 張大川 ² ,  邱慧 ¹

1. ;
2. ;

Corresponding?author：

邱慧, Email: naturehui@126.com

Keywords：

肺動脈肉瘤; 肺栓塞

DOI：

10.7507/1671-6205.202202022

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目的探討肺動脈肉瘤（pulmonary artery sarcoma，PAS）的臨床表現、影像學表現、病理學特點及鑒別診斷，提高臨床醫生對 PAS 的診治水平。方法分析1 例經病理活檢確診的 PAS 患者的臨床資料并復習相關文獻。結果患者男，72 歲，因“咳嗽、胸悶半年”于 2018 年 2 月 27 日首次入院。既往體健。查體無特殊。胸部 CT 示左肺下葉見多發結節狀高密度影，左肺動脈主干及分支內見斑塊狀低密度充盈影。診斷肺栓塞，予低分子肝素抗凝等治療后好轉出院。2018 年 4 月患者癥狀加重，復查 CT 肺動脈造影示左肺門增大，左肺動脈擴張，左肺動脈干管腔內巨大充盈缺損，肺動脈分叉處、左肺動脈多個分支及左心房亦可見多發充盈缺損，與首次入院時病灶相比明顯進展。正電子發射斷層顯像檢查示左肺動脈主干管腔擴張伴腔內低密度影，氟代脫氧葡萄糖代謝減低，左肺下葉結節樣放射性濃聚影，SUVmax 10.3。行經肺動脈介入抽吸取栓術，病理確診為 PAS。以“pulmonary artery sarcoma”“肺動脈肉瘤”“PAS”“pulmonary artery intimal sarcoma”“肺動脈內膜肉瘤”“PAIS”“pulmonary thromboembolism”“肺栓塞”“PTE”為關鍵詞對國內外文獻進行檢索，共檢索到相關文獻 10 篇，涉及 506 例患者，其中男 265 例，女 241 例。PAS 患者的主要癥狀為咳嗽和胸悶。CT 肺動脈造影見肺動脈主干充盈缺損，可累及左右肺動脈及其分支。PAS 大都經手術確診。病理見黏液背景中大量類似成纖維細胞或肌成纖維細胞的梭形細胞肉瘤，核異型性及有絲分裂像明顯。免疫組織化學檢測無特異性標志物，波形蛋白強陽性，CD31、CD34、Fli-1 不同程度陽性。手術切除是其主要的治療手段，預后差。結論 PAS 是罕見的肺血管系統的惡性腫瘤。患者的臨床表現、影像學檢查和病理免疫組織化學檢測無明顯特異性，常被誤診為 PTE。及時診斷和早期治療是延長患者生存時間的關鍵。

Citation： 楊寵玉, 王亞麗, 李志英, 張秋娣, 張大川, 邱慧. 肺動脈肉瘤一例并文獻復習. 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, 劉盛, 鄧隆, 等. 17 例肺動脈肉瘤的外科治療. 中國循環雜志, 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.	周錦桃, 葉伶. 12 例肺動脈肉瘤患者的臨床分析. 臨床腫瘤學雜志, 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 prim?re 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.	梁穎, 米玉紅, 高元明, 等. 肺動脈肉瘤 8 例臨床分析. 中國呼吸與危重監護雜志, 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.	姜維, 楊媛華, 李一丹, 等. 肺動脈肉瘤的超聲心動圖表現并文獻回顧. 中國超聲醫學雜志, 2019, 35(11): 1048-1051.
22.	陳兢兢, 鐘志成, 李靜. 原發性肺動脈肉瘤的診療現狀. 中華結核和呼吸雜志, 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.	甘輝立, 張健群, 黃小勇, 等. 肺動脈CT造影檢查對肺動脈肉瘤鑒別診斷的意義. 心肺血管病雜志, 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.	劉陽祥, 王進, 吳曉虹, 等. 肺動脈肉瘤 4 例臨床分析并文獻復習. 國際呼吸雜志, 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, 劉盛, 鄧隆, 等. 17 例肺動脈肉瘤的外科治療. 中國循環雜志, 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. 周錦桃, 葉伶. 12 例肺動脈肉瘤患者的臨床分析. 臨床腫瘤學雜志, 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 prim?re 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. 梁穎, 米玉紅, 高元明, 等. 肺動脈肉瘤 8 例臨床分析. 中國呼吸與危重監護雜志, 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. 姜維, 楊媛華, 李一丹, 等. 肺動脈肉瘤的超聲心動圖表現并文獻回顧. 中國超聲醫學雜志, 2019, 35(11): 1048-1051.
22. 陳兢兢, 鐘志成, 李靜. 原發性肺動脈肉瘤的診療現狀. 中華結核和呼吸雜志, 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. 甘輝立, 張健群, 黃小勇, 等. 肺動脈CT造影檢查對肺動脈肉瘤鑒別診斷的意義. 心肺血管病雜志, 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. 劉陽祥, 王進, 吳曉虹, 等. 肺動脈肉瘤 4 例臨床分析并文獻復習. 國際呼吸雜志, 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

肺動脈肉瘤一例并文獻復習

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