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[1]刘小兵,傅 优,陈 佳,等.肺上皮样血管内皮瘤7例报告并文献复习[J].中华肺部疾病杂志,2025,(01):92-97.[doi:10.3877/cma.j.issn.1674-6902.2025.01.015 ]
　Liu Xiaobing,Fu You,Chen Jia,et al.Case reports and literature review of seven cases of pulmonary epithelioid hemangioendothelioma[J].,2025,(01):92-97.[doi:10.3877/cma.j.issn.1674-6902.2025.01.015 ]
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肺上皮样血管内皮瘤7例报告并文献复习(PDF)

《中华肺部疾病杂志》[ISSN:1006-6977/CN:61-1281/TN]

卷:
期数:
2025年01期
页码:
92-97
栏目:
论著
出版日期:
2025-02-25

文章信息/Info

Title:
Case reports and literature review of seven cases of pulmonary epithelioid hemangioendothelioma
作者:
刘小兵1; 傅 优2; 陈 佳3; 刘权兴1; 戴纪刚1
400037 重庆,陆军(第三)军医大学第二附属医院胸外科1
400037 重庆,陆军(第三)军医大学第二附属医院病理科
400037 重庆,陆军(第三)军医大学第二附属医院放射科3
Author(s):
Liu Xiaobing1;  Fu You2;  Chen Jia3;  Liu Quanxing1;  Dai Jigang1.
1Department of Thoracic Surgery, Xinqiao Hospital, Army Medical University(Third Military Medical University), Chongqing, China; 2Department of Pathology, Xinqiao Hospital, Army Medical University(Third Military Medical University),
关键词:
肺上皮样血管内皮瘤;  支气管肺癌;  文献复习
Keywords:
Pulmonary epithelioid hemangioendothelioma;  Bronchogenic carcinoma;  Literature revien
分类号:
R734.2
DOI:
10.3877/cma.j.issn.1674-6902.2025.01.015
摘要:
目的 肺上皮样血管内皮瘤(pulmonary epithelioid hemangioendothelioma, PEH)为罕见低度恶性血管源性肿瘤,发病率约0.01/10万。通过系统分析我院诊治病例结合文献复习,探讨肺上皮样血管内皮瘤的临床表现、诊断要点及治疗策略,为临床治疗提供循证依据。方法 选择2019年4月至2024年6月我院收治的7例肺上皮样血管内皮瘤患者。收集人口学特征、临床表现、实验室指标、影像学特征、手术记录、组织病理学及随访数据。运用PubMed、CNKI数据库进行文献检索,筛选近10年相关文献,构建多维分析框架及文献复习。 结果 7例患者中男5例,女2例,年龄31～60岁,平均年龄(47.86±10.32)岁,临床表现呈非特异性:慢性咳嗽2例(28.57%),伴胸痛1例(14.28%),体检发现4例(57.14%)。影像学特征:CT显示小结节(<2 cm)5例(71.43%),单发肿块、双肺结节各1例(14.28%)。7例行手术治疗,胸腔镜楔形切除2例(28.57%),肺段切除1例(14.28%),肺叶切除4例(57.14%)。免疫组化病理确诊:CD31 7例阳性、CD34 7例阳性。中位随访27.10(1～63)个月,随访结果肺上皮血管内皮瘤患者显示总体预后良好。结论 肺上皮样血管内皮瘤诊断需结合临床-影像-病理三联征,免疫组化检测具有临床意义。外科手术是治疗肺上皮血管内皮瘤患者的核心手段,推荐胸腔镜微创技术为首选。无法完全切除或多发灶病例,建议采取个体化综合治疗策略,包括化疗、放疗、靶向治疗及密切随访。
Abstract:
Objective Pulmonary epithelioid hemangioendothelioma(PEH)is a rare, low-grade malignant vascular tumor with an incidence rate of approximately 0.01 per 100,000 individuals. To provide an evidence-based foundation for clinical decision-making by systematically analyzing the clinical manifestations, diagnostic features, and treatment strategies of PEH through a review of cases treated at our hospital and a literature review. Methods This study retrospectively included seven patients with PEH admitted to our hospital from April 2019 to June 2024. Data on demographic characteristics, clinical manifestations, laboratory findings, imaging features, surgical records, pathological reports, and follow-up information were collected via the electronic medical record system. Additionally, a literature search was conducted using PubMed and CNKI databases to screen relevant articles published in the last decade. A multidimensional analytical framework was constructed for in-depth review and discussion. Results The patients were 5 males and 2 females, ranging in age from 31 to 60 years, with an average age of(47.86±10.32)years. Clinical manifestations were non-specific: chronic cough was present in two cases(28.57%), chest pain in one case(14.28%), and incidental findings during physical examination in four cases(57.14%). Imaging features included small nodules(<2 cm)in five cases(71.43%), solitary masses in one case(14.28%), and bilateral nodules in one case(14.28%). All cases underwent surgical treatment, with thoracoscopic wedge resection in two cases(28.57%), segmentectomy in one case(14.28%), and lobectomy in four cases(57.14%). Pathological confirmation relied on immunohistochemistry: CD31(positive in 7/7)and CD34(positive in 7/7). The median follow-up duration was 27.10 months(1-63 months), with long-term follow-up indicating a generally favorable prognosis for all patients. Conclusion The diagnosis of pulmonary epithelioid hemangioendothelioma requires an integration of clinical, imaging, and pathological findings, with immunohistochemistry playing a crucial role. Surgical resection remains the cornerstone of curative treatment, with thoracoscopic minimally invasive techniques recommended as the preferred approach. For cases that cannot be completely resected or are multifocal, an individualized comprehensive treatment strategy, including radiotherapy, targeted therapy, and close follow-up, is suggested.

参考文献/References:

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]. J Thorac Oncol, 2015, 10(9): 1243-1260.
2 Onishi Y, Kusumoto M, Goto Y, et al. Epithelioid hemangioendothelioma of the lung: CT findings and clinical course of 35 cases [J]. Jpn J Clin Oncol, 2020, 50(10): 1195-1200.
3 Sardaro A, Bardoscia L, Petruzzelli MF, et al. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor [J]. Oncol Rev, 2014, 8(2): 259.
4 Aung TT, Chu A, Kondapi D, et al. A case of pulmonary epithelioid hemangioendothelioma with literature review [J]. Case Rep Oncol Med, 2020, 2020: 8048056.
5 Botnaru V, Munteanu O, Vataman V, et al. [Pulmonary epithelioid hemangioendothelioma-a case report][J]. Pneumologia, 2008, 57(4): 234-235, 237-238.
6 Mardani P, Shahriarirad R, Nekooeian M, et al. Pleural epithelioid hemangioendothelioma in a 39-Year-old female: a case report [J]. J Cardiothorac Surg, 2024, 19(1): 118.
7 Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: a vascular tumor often mistaken for a carcinoma[J]. Cancer, 1982, 50(5): 970-981.
8 Paulson KG, Ravi V, Rubin BP, et al. Incidence, demographics, and survival of malignant hemangioendothelioma in the United States [J]. Cancer Med, 2023, 12(14): 15101-15106.
9 Dermawan JK, Azzato EM, Billings S D, et al. YAP1-TFE3-fused hemangioendothelioma: a multi-institutional clinicopathologic study of 24 genetically-confirmed cases [J]. Mod Pathol, 2021, 34(12): 2211-2221.
10 Li S, Dermawan JK, Seavey CN, et al. Epithelioid hemangioendothelioma(EHE)with WWTR1:TFE3 gene fusion, a novel fusion variant [J]. Genes Chromosomes Cancer, 2024, 63(2): e23226.
11 Celikel C, Yumuk PF, Basaran G, et al. Epithelioid hemangioendothelioma with multiple organ involvement [J]. Apmis, 2007, 115(7): 881-888.
12 Wu R, Xia X, Hu F, et al. Case Report:(18)F-FDG PET/CT demonstrating malignant spread of a pulmonary epithelioid hemangioendothelioma [J]. Front Med(Lausanne), 2022, 9: 862690.
13 Fan Y, Wang F, Li S, et al. Pleural epithelioid hemangioendothelioma: A case report and literature review [J]. J Natl Med Assoc, 2016, 108(2): 124-129.
14 Kim EY, Kim TS, Han J, et al. Thoracic epithelioid hemangioendothelioma: imaging and pathologic features [J]. Acta Radiol, 2011, 52(2): 161-166.
15 Gong J, Tian F, Wang Q, et al. Case report: Rare epithelioid hemangioendothelioma occurs in both main bronchus and lung [J]. Front Med(Lausanne), 2022, 9: 1066870.
16 Mao X, Liang Z, Chibhabha F, et al. Clinico-radiological features and next generation sequencing of pulmonary epithelioid hemangioendothelioma: A case report and review of literature [J]. Thorac Cancer, 2017, 8(6): 687-692.
17 王颖奕, 梁远凤, 王光宪. 肺上皮样血管内皮瘤的CT特征及临床分析[J/CD]. 中华肺部疾病杂志(电子版), 2021, 14(4): 462-465.
18 Yi GY, Kim YK, Kim KC, et al. Pulmonary multinodular epithelioid hemangioendothelioma with mixed progression and spontaneous regression during a 7-year follow-up: A case report and review of imaging findings[J]. J Korean Soc Radiol, 2022, 83(4): 958-964.
19 Mesquita RD, Sousa M, Trinidad C, et al. New insights about pulmonary epithelioid hemangioendothelioma: review of the literature and two case reports[J]. Case Rep Radiol, 2017, 2017: 5972940.
20 �瘙 塁 enses V, Kaplan Ï, Kaya F, et al. 68 Ga-PSMA Versus 18 F-FDG PET/CT in the evaluation of epithelioid hemangioendothelioma[J]. Clin Nucl Med, 2024, 49(9): e464-e467.
21 Jang YC, Hung WC, Su TC, et al. Primary pulmonary epithelioid hemangioendothelioma[J]. BMJ Case Rep, 2023, 16(9): e254915.
22 Graça LL, Almeida Cunha S, Lopes RS, et al. Thoracic epithelioid hemangioendothelioma: clinical demonstration and therapeutic procedures[J]. Port J Card Thorac Vasc Surg, 2022, 29(2): 39-44.
23 ochowski M, Rbowski M, Jesionek-kupnicka D, et al. Pulmonary epithelioid hemangioendothelioma imitating lung cancer[J]. Kardiochir Torakochirurgia Pol, 2017, 14(3): 209-210.
24 罗 虎, 邓才霞, 段江洁, 等. 肺上皮样血管内皮瘤6例临床分析并文献复习[J]. 第三军医大学学报, 2015, 37(24): 2472-2476.
25 Jirasirinuphan P, Chang AL, Deepak A, et al. Delayed metastatic pulmonary epithelioid hemangioendothelioma of the femoral vessels: case report and literature review[J]. AME Case Rep, 2023, 7: 1.
26 Pálföldi r, Radács M, Csada E, et al. Pulmonary epithelioid haemangioendothelioma studies in vitro and in vivo: new diagnostic and treatment methods[J]. In Vivo, 2013, 27(2): 221-225.
27 Guo W, Zhou D, Huang H, et al. Successful chemotherapy with continuous immunotherapy for primary pulmonary endovascular epithelioid hemangioendothelioma: A case report [J]. Medicine(Baltimore), 2023, 102(7): e32914.
28 Schmidt M, Mattern S, Singer S, et al. NOTCH3 missense mutations as predictor of long-term response to gemcitabine in a patient with epithelioid hemangioendothelioma [J]. J Cancer Res Clin Oncol, 2023, 149(9): 6753-6757.
29 Kpodonu J, Tshibaka C, Massad MG. The importance of clinical registries for pulmonary epithelioid hemangioendothelioma [J]. Chest, 2005, 127(5): 1870-1871.
30 Blay JY, Piperno-neumann S, Watson S, et al. Epithelioid hemangio-endothelioma(EHE)in NETSARC: The nationwide series of 267 patients over 12 years [J]. Eur J Cancer, 2023, 192: 113262.

备注/Memo

备注/Memo:
基金项目: 陆军军医大学第二附属医院青博项目(2022YQB043)
重庆市技术创新与应用发展重点专项项目(CSTB2022TIAD-CUX0019)
通信作者: 戴纪刚, Email: daijigang@tmmu.edu.cn
更新日期/Last Update: 2025-02-20