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。

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 nonspecific： 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.