胸膜上皮樣血管內皮瘤一例報道并文獻復習_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

束衛燕 ^1,2 , 張安梅 ¹ ,  王凱歌 ²

1. ;
2. ;

Corresponding?author：

王凱歌, Email: youjiangbuyi@163.com

Keywords：

胸膜腫瘤; 上皮樣血管內皮瘤; 基因檢測; 靶向治療; 罕見病

DOI：

10.7507/1671-6205.202604010

Video：

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目的探討胸膜上皮樣血管內皮瘤（epithelioid hemangioendothelioma，EHE）的臨床特征，提高臨床對胸膜EHE的識別，歸納病例數據，為后續EHE的相關研究提供數據支持。方法梳理四川大學華西醫院收治的1例胸膜EHE的診療資料，結合文獻，探討胸膜EHE的病理特征及治療方案研究進展情況。結果本例患者46歲，女性。因“咳嗽、氣促4⁺個月，腹痛2⁺個月”就診。胸部增強CT示右肺多發結節，右側胸腔包裹性積液，右側胸膜增厚伴強化。CT引導下胸膜活檢，病理結果顯示梭形細胞腫瘤；免疫組化：CD31（±）、CD34（+）、ERG（+）、FLI-1（+）、WT1（±）、D2-40（部分+）；FISH檢測結果：FISH-CAMTA1易位（+）、FISH-TFE3易位（–）、FISH-p16（–）。確診為胸膜EHE，WWTR1-CAMTA1融合型，伴腹膜浸潤，肺內轉移可能。患者在當地醫院接受紫杉醇120 mg，d1方案化療，出現消化道出血，遂放棄治療，確診4個月后去世。結論 EHE屬罕見腫瘤，胸膜部位EHE更為罕見，且侵襲性更強。胸膜EHE臨床表現缺乏特異性，確診依賴病理形態、免疫組化及FISH/NGS基因檢測的聯合應用。其治療尚無統一標準，局限性病變首選手術切除，可改善生存率；而對于進展、轉移病變者多行全身治療，其中針對TAZ-CAMTA1-TEAD軸的靶向研究是具有潛力的新方向。

1.	Liu Z, He S. Epithelioid Hemangioendothelioma: incidence, mortality, prognostic factors, and survival analysis using the surveillance, epidemiology, and end results database[J]. J Oncol, 2022, 2022: 2349991.
2.	Weadick CS, Alshibany A, Michael MJ, et al. Incidence, prevalence and treatment of patients with epithelioid haemangioendothelioma (EHE) in Canada: a Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) and Multi-pronged Canadian Research in Epithelioid Haemangioendothelioma (PRO_CARE EHE) study[J]. Eur J Cancer, 2025, 231: 116100.
3.	Zhang S, Liang Y, Yang Y, et al. Clinicopathological features, risk model and prognosis of 115 cases of epithelioid hemangioendothelioma: a single-center study[J]. Front Oncol, 2025, 15: 1577968.
4.	Rezvani A, Shahriarirad R, Erfani A, et al. Primary malignant epithelioid hemangioendothelioma of the pleura: a review and report of a novel case[J]. Clin Case Rep, 2022, 10(8): e6211.
5.	Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma[J]. Cancer, 1982, 50(5): 970-981.
6.	Stacchiotti S, Miah AB, Frezza AM, et al. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts[J]. ESMO Open, 2021, 6(3): 100170.
7.	姜楠, 胡春洪, 胡粟等. 肺上皮樣血管內皮瘤影像表現及臨床特點[J]. 實用放射學雜志, 2023, 39(08): 1241-1243.
8.	Pateras IS, Kostopanagiotou K, Samaras MG, et al. Rare cancer with primary pleural epithelioid hemangioendothelioma diagnosed by thoracoscopic biopsy achieving disease control after 16 months: case report and literature review[J]. Front Pharmacol, 2024, 15: 1482154.
9.	隋成亮, 沈艷瑩, 劉澤兵. 上皮樣血管內皮瘤12例臨床病理學分析[J]. 上海交通大學學報(醫學版), 2025, 45(7): 892-899.
10.	王雙雙, 孫怡, 李惠, 等. 上皮樣血管內皮瘤臨床病理特征分析[J]. 診斷病理學雜志, 2026, 33(2): 180-185.
11.	謝曉楓, 黃焰, 郭俊紅, 等. 易被誤診的胸膜原發上皮樣血管內皮瘤五例臨床病理學分析[J]. 中華病理學雜志, 2020, 49(12): 1288-1293.
12.	李雯, 李俊康, 鄭昕, 等. 肺原發性上皮樣血管內皮瘤7例臨床病理分析[J]. 中華腫瘤雜志, 2025, 47(03): 269-274.
13.	Mutsaers SE, Birnie K, Lansley S, et al. Mesothelial cells in tissue repair and fibrosis[J]. Front Pharmacol, 2015, 6: 113.
14.	Antonescu CR, Le Loarer F, Mosquera JM, et al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma[J]. Genes Chromosomes Cancer, 2013, 52(8): 775-784.
15.	Driskill JH, Zheng Y, Wu BK, et al. WWTR1(TAZ)-CAMTA1 reprograms endothelial cells to drive epithelioid hemangioendothelioma[J]. Genes Dev, 2021, 35(7-8): 495-511.
16.	Seavey CN, Pobbati AV, Hallett A, et al. WWTR1(TAZ)-CAMTA1 gene fusion is sufficient to dysregulate YAP/TAZ signaling and drive epithelioid hemangioendothelioma tumorigenesis[J]. Genes Dev, 2021, 35(7-8): 512-527.
17.	Abdelmogod A, Papadopoulos L, Riordan S, et al. A matched molecular and clinical analysis of the epithelioid haemangioendothelioma cohort in the Stafford Fox Rare Cancer Program and contextual literature review[J]. Cancers (Basel), 2023, 15(17): 4378.
18.	Stacchiotti S, Simeone N, Lo Vullo S, et al. Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: a case-series analysis within the Italian Rare Cancer Network[J]. Cancer, 2021, 127(4): 569-576.
19.	Schuetze S, Ballman KV, Ganjoo KN, et al. P10015/SARC033: A phase 2 trial of trametinib in patients with advanced epithelioid hemangioendothelioma (EHE)[J]. J Clin Oncol, 2021, 39(15_suppl): 11503-11503.
20.	Burkhard-Meier A, Rechenauer VV, Jurinovic V, et al. First-line pazopanib in patients with metastatic epithelioid hemangioendothelioma: a retrospective single-center analysis[J]. J Cancer Res Clin Oncol, 2025, 151(4): 150.
21.	Kanai R, Pobbati AV, Li S, et al. The TAZ-CAMTA1 Fusion Protein Promotes Tumorigenesis via Connective Tissue Growth Factor and Ras-MAPK Signaling in Epithelioid Hemangioendothelioma[J]. Clin Cancer Res, 2022, 28(14): 3116-3126.

1. Liu Z, He S. Epithelioid Hemangioendothelioma: incidence, mortality, prognostic factors, and survival analysis using the surveillance, epidemiology, and end results database[J]. J Oncol, 2022, 2022: 2349991.
2. Weadick CS, Alshibany A, Michael MJ, et al. Incidence, prevalence and treatment of patients with epithelioid haemangioendothelioma (EHE) in Canada: a Canadian Sarcoma Research and Clinical Collaboration (CanSaRCC) and Multi-pronged Canadian Research in Epithelioid Haemangioendothelioma (PRO_CARE EHE) study[J]. Eur J Cancer, 2025, 231: 116100.
3. Zhang S, Liang Y, Yang Y, et al. Clinicopathological features, risk model and prognosis of 115 cases of epithelioid hemangioendothelioma: a single-center study[J]. Front Oncol, 2025, 15: 1577968.
4. Rezvani A, Shahriarirad R, Erfani A, et al. Primary malignant epithelioid hemangioendothelioma of the pleura: a review and report of a novel case[J]. Clin Case Rep, 2022, 10(8): e6211.
5. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma[J]. Cancer, 1982, 50(5): 970-981.
6. Stacchiotti S, Miah AB, Frezza AM, et al. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts[J]. ESMO Open, 2021, 6(3): 100170.
7. 姜楠, 胡春洪, 胡粟等. 肺上皮樣血管內皮瘤影像表現及臨床特點[J]. 實用放射學雜志, 2023, 39(08): 1241-1243.
8. Pateras IS, Kostopanagiotou K, Samaras MG, et al. Rare cancer with primary pleural epithelioid hemangioendothelioma diagnosed by thoracoscopic biopsy achieving disease control after 16 months: case report and literature review[J]. Front Pharmacol, 2024, 15: 1482154.
9. 隋成亮, 沈艷瑩, 劉澤兵. 上皮樣血管內皮瘤12例臨床病理學分析[J]. 上海交通大學學報(醫學版), 2025, 45(7): 892-899.
10. 王雙雙, 孫怡, 李惠, 等. 上皮樣血管內皮瘤臨床病理特征分析[J]. 診斷病理學雜志, 2026, 33(2): 180-185.
11. 謝曉楓, 黃焰, 郭俊紅, 等. 易被誤診的胸膜原發上皮樣血管內皮瘤五例臨床病理學分析[J]. 中華病理學雜志, 2020, 49(12): 1288-1293.
12. 李雯, 李俊康, 鄭昕, 等. 肺原發性上皮樣血管內皮瘤7例臨床病理分析[J]. 中華腫瘤雜志, 2025, 47(03): 269-274.
13. Mutsaers SE, Birnie K, Lansley S, et al. Mesothelial cells in tissue repair and fibrosis[J]. Front Pharmacol, 2015, 6: 113.
14. Antonescu CR, Le Loarer F, Mosquera JM, et al. Novel YAP1-TFE3 fusion defines a distinct subset of epithelioid hemangioendothelioma[J]. Genes Chromosomes Cancer, 2013, 52(8): 775-784.
15. Driskill JH, Zheng Y, Wu BK, et al. WWTR1(TAZ)-CAMTA1 reprograms endothelial cells to drive epithelioid hemangioendothelioma[J]. Genes Dev, 2021, 35(7-8): 495-511.
16. Seavey CN, Pobbati AV, Hallett A, et al. WWTR1(TAZ)-CAMTA1 gene fusion is sufficient to dysregulate YAP/TAZ signaling and drive epithelioid hemangioendothelioma tumorigenesis[J]. Genes Dev, 2021, 35(7-8): 512-527.
17. Abdelmogod A, Papadopoulos L, Riordan S, et al. A matched molecular and clinical analysis of the epithelioid haemangioendothelioma cohort in the Stafford Fox Rare Cancer Program and contextual literature review[J]. Cancers (Basel), 2023, 15(17): 4378.
18. Stacchiotti S, Simeone N, Lo Vullo S, et al. Activity of sirolimus in patients with progressive epithelioid hemangioendothelioma: a case-series analysis within the Italian Rare Cancer Network[J]. Cancer, 2021, 127(4): 569-576.
19. Schuetze S, Ballman KV, Ganjoo KN, et al. P10015/SARC033: A phase 2 trial of trametinib in patients with advanced epithelioid hemangioendothelioma (EHE)[J]. J Clin Oncol, 2021, 39(15_suppl): 11503-11503.
20. Burkhard-Meier A, Rechenauer VV, Jurinovic V, et al. First-line pazopanib in patients with metastatic epithelioid hemangioendothelioma: a retrospective single-center analysis[J]. J Cancer Res Clin Oncol, 2025, 151(4): 150.
21. Kanai R, Pobbati AV, Li S, et al. The TAZ-CAMTA1 Fusion Protein Promotes Tumorigenesis via Connective Tissue Growth Factor and Ras-MAPK Signaling in Epithelioid Hemangioendothelioma[J]. Clin Cancer Res, 2022, 28(14): 3116-3126.

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