原發性肺滑膜肉瘤61例病例分析_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

黃艷生 ¹ , 黃進寶 ² , 江梅琴 ² , 祝雪麗 ¹ , 花聰 ¹ ,  李紅艷 ¹

1. ;
2. ;

Corresponding?author：

李紅艷, Email: 1353626039@qq.com

Keywords：

滑膜肉瘤; 手術; 預后

DOI：

10.7507/1671-6205.202601067

Video：

Export PDF Favorites Scan Get Citation

Abstract Full text Figures/Tables Video References Cited by

目的探討原發性肺滑膜肉瘤（primary pulmonary synovial sarcoma，PPSS）的臨床特征，以提高對本病的認識。方法通過中國知網主題搜索“原發性肺滑膜肉瘤”，共納入信息資料相對完整的“原發性肺滑膜肉瘤”病例61例。對患者的一般情況、臨床癥狀、影像表現、病理特征及治療轉歸進行分析。結果 61例PPSS患者中男性占63.9%（39/61），女性占36.1%（22/61）；年齡13～78歲，平均年齡46.4歲；臨床癥狀以咳嗽（40/61，65.6%）、胸痛（26/61，42.6%）、氣喘（14/61，23.0%）、咯血（14/61，23.0%）為主。PPSS肺內表現為圓形或類圓形的肺結節，肺結節以單發結節多見（39/40，97.5%），肺結節分布以左下（15/61，24.6%）、右上（15/61，24.6%）常見。病理表現為彌漫性生長的梭形細胞腫瘤。治療上以手術治療（40/49，81.6%）、化療（16/33，48.5%）、放療（6/34，17.6%）為主。手術+化療組與手術無化療組的無復發生存期及總生存期差異無統計學意義（P>0.05）。結論 PPSS以男性略多見，臨床癥狀表現為咳嗽、胸痛、氣喘、咯血。胸部CT多表現為單發結節，病理可見彌漫性梭形腫瘤細胞，免疫組化常見Vim、EMA、CD99、CK、BCL-2陽性。治療上目前多以手術治療為主，放化療為輔。手術患者聯合化療可能不會給PPSS帶來獲益。

Citation： 黃艷生, 黃進寶, 江梅琴, 祝雪麗, 花聰, 李紅艷. 原發性肺滑膜肉瘤61例病例分析. Chinese Journal of Respiratory and Critical Care Medicine, 2026, 25(7): 519-522. doi: 10.7507/1671-6205.202601067 Copy

1.	Spillane AJ, A'Hern R, Judson IR, et al. Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment. J Clin Oncol, 2000, 18: 3794-3803.
2.	Keel SB, Bacha E, Mark EJ, et al. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol, 1999, 12(12): 1124-31.
3.	Etienne-Mastroianni B, Falchero L, Chalabreysse L, et al. Primary sarcomas of the lung. A clinicopathologic study of 12 cases. Lung Cancer, 2002, 38: 283.
4.	Mankin HJ, Hornicek FJ. Diagnosis, classification, and management of soft tissue sarcomas. Cancer Control, 2005, 12: 5-21.
5.	Roy PP, Das A, Sarkar A, et al. A primary synovial sarcoma of lung. N Am J Med Sci, 2012, 4(5): 241e3.
6.	Al-Ani Z, Naqvi J, Oh TC. Primary pleuropulmonary synovial sarcoma mimicking a carcinoid tumor: case report and literature review. Radiol Case Rep, 2016, 11: 45-49.
7.	黃艷生, 李紅艷, 黃進寶, 等. ECMO輔助下手術治療單側左肺復發性肺滑膜肉瘤1例. 中國腫瘤臨床, 2024, 51(20): 1073-1075.
8.	Kumar R, Menon S, Desai SB, et al. Primary endobronchial synovial sarcoma conffrmed by SYT-SSX1 fusion gene transcript by reverse transcriptase polymerase chain reaction. Indian J Pathol Microbiol, 2009, 52(4): 520-523.
9.	Rahmaniar D, Maranatha D. The early-stage of primary pulmonary synovial sarcoma: a case report. Int J Surg Case Rep, 2022, 95: 107251.
10.	Panigrahi MK, Pradhan G, Sahoo N, et al. Primary pulmonary synovial sarcoma: a reappraisal. J Cancer Res Ther, 2018, 14(3): 481-489.
11.	王璇, 程凱, 吳楠, 等. SS18-SSX融合特異性抗體和C-末端SSX抗體在滑膜肉瘤中的表達及診斷價值. 中華病理學雜志, 2021, 50(7): 740-744.
12.	Dimitriadis E, Rontogianni D, Kyriazoglou A, et. al. Novel SYT-SSX fusion transcript variants in synovial sarcoma. Cancer Genet Cytogenet, 2009, 195(1): 54-58.
13.	李思平, 劉麗平, 周榮. 原發性胸膜滑膜肉瘤1例. 臨床肺科雜志, 2023, 28(9): 1453-1456.
14.	Wu D, Zhang H, Li X, Liu J, et al. Whole-exome sequencing in primary pulmonary synovial sarcoma misdiagnosed as mesothelioma: a case report and literature review. Thorac Cancer, 2023, 14(16): 1520-1529.
15.	Meyer M, Seetharam M. First-line therapy for metastatic soft tissue sarcoma. Curr Treat Options Oncol, 2019, 20(1): 6.
16.	Vlenterie M, Jones RL, van der Graaf WT. Synovial sarcoma diagnosis and management in the era of targeted therapies. Curr Opin Oncol, 2015, 27(4): 316-322.
17.	Kagawa Y, Kitaguchi S, Konishi H, et al. Primary advanced pulmonary synovial sarcoma treated with a multidisciplinary approach. Int Cancer Conf J, 2020, 9(2): 72-76.
18.	Wakamatsu T, Naka N, Sasagawa S, et al. Deflection of vascular endothelial growth factor action by SS18-SSX and composite vascular endothelial growth factor- and chemokine (C-X-C motif) receptor 4-targeted therapy in synovial sarcoma. Cancer Sci, 2014, 105: 1124-1134.
19.	van der Graaf WT, Blay JY, Chawla SP, et al. EORTC Soft Tissue and Bone Sarcoma Group; PALETTE study group. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet, 2012, 379(9829): 1879-1886.
20.	Wang W, Guo J, Bao M, et al. Primary pulmonary synovial sarcoma. JTCVS Open, 2022, 10: 404-414.
21.	Guo C, Liu C, Pu Q, et al. Occult primary pulmonary synovial sarcoma presenting as recurrent spontaneous pneumothorax and explosive progression. Thorac Cancer, 2017, 8(2): 121-123.

1. Spillane AJ, A'Hern R, Judson IR, et al. Synovial sarcoma: a clinicopathologic, staging, and prognostic assessment. J Clin Oncol, 2000, 18: 3794-3803.
2. Keel SB, Bacha E, Mark EJ, et al. Primary pulmonary sarcoma: a clinicopathologic study of 26 cases. Mod Pathol, 1999, 12(12): 1124-31.
3. Etienne-Mastroianni B, Falchero L, Chalabreysse L, et al. Primary sarcomas of the lung. A clinicopathologic study of 12 cases. Lung Cancer, 2002, 38: 283.
4. Mankin HJ, Hornicek FJ. Diagnosis, classification, and management of soft tissue sarcomas. Cancer Control, 2005, 12: 5-21.
5. Roy PP, Das A, Sarkar A, et al. A primary synovial sarcoma of lung. N Am J Med Sci, 2012, 4(5): 241e3.
6. Al-Ani Z, Naqvi J, Oh TC. Primary pleuropulmonary synovial sarcoma mimicking a carcinoid tumor: case report and literature review. Radiol Case Rep, 2016, 11: 45-49.
7. 黃艷生, 李紅艷, 黃進寶, 等. ECMO輔助下手術治療單側左肺復發性肺滑膜肉瘤1例. 中國腫瘤臨床, 2024, 51(20): 1073-1075.
8. Kumar R, Menon S, Desai SB, et al. Primary endobronchial synovial sarcoma conffrmed by SYT-SSX1 fusion gene transcript by reverse transcriptase polymerase chain reaction. Indian J Pathol Microbiol, 2009, 52(4): 520-523.
9. Rahmaniar D, Maranatha D. The early-stage of primary pulmonary synovial sarcoma: a case report. Int J Surg Case Rep, 2022, 95: 107251.
10. Panigrahi MK, Pradhan G, Sahoo N, et al. Primary pulmonary synovial sarcoma: a reappraisal. J Cancer Res Ther, 2018, 14(3): 481-489.
11. 王璇, 程凱, 吳楠, 等. SS18-SSX融合特異性抗體和C-末端SSX抗體在滑膜肉瘤中的表達及診斷價值. 中華病理學雜志, 2021, 50(7): 740-744.
12. Dimitriadis E, Rontogianni D, Kyriazoglou A, et. al. Novel SYT-SSX fusion transcript variants in synovial sarcoma. Cancer Genet Cytogenet, 2009, 195(1): 54-58.
13. 李思平, 劉麗平, 周榮. 原發性胸膜滑膜肉瘤1例. 臨床肺科雜志, 2023, 28(9): 1453-1456.
14. Wu D, Zhang H, Li X, Liu J, et al. Whole-exome sequencing in primary pulmonary synovial sarcoma misdiagnosed as mesothelioma: a case report and literature review. Thorac Cancer, 2023, 14(16): 1520-1529.
15. Meyer M, Seetharam M. First-line therapy for metastatic soft tissue sarcoma. Curr Treat Options Oncol, 2019, 20(1): 6.
16. Vlenterie M, Jones RL, van der Graaf WT. Synovial sarcoma diagnosis and management in the era of targeted therapies. Curr Opin Oncol, 2015, 27(4): 316-322.
17. Kagawa Y, Kitaguchi S, Konishi H, et al. Primary advanced pulmonary synovial sarcoma treated with a multidisciplinary approach. Int Cancer Conf J, 2020, 9(2): 72-76.
18. Wakamatsu T, Naka N, Sasagawa S, et al. Deflection of vascular endothelial growth factor action by SS18-SSX and composite vascular endothelial growth factor- and chemokine (C-X-C motif) receptor 4-targeted therapy in synovial sarcoma. Cancer Sci, 2014, 105: 1124-1134.
19. van der Graaf WT, Blay JY, Chawla SP, et al. EORTC Soft Tissue and Bone Sarcoma Group; PALETTE study group. Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet, 2012, 379(9829): 1879-1886.
20. Wang W, Guo J, Bao M, et al. Primary pulmonary synovial sarcoma. JTCVS Open, 2022, 10: 404-414.
21. Guo C, Liu C, Pu Q, et al. Occult primary pulmonary synovial sarcoma presenting as recurrent spontaneous pneumothorax and explosive progression. Thorac Cancer, 2017, 8(2): 121-123.

Previous Article
Meta-analysis of factors influencing oral mucosal pressure injuries in ICU patients with endotracheal intubation
Next Article
肺動態順應性與超聲聯合滴定PEEP策略

Chinese Journal of Respiratory and Critical Care Medicine

原發性肺滑膜肉瘤61例病例分析

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content