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 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.

Key words: Pulmonary epithelioid hemangioendothelioma, Bronchogenic carcinoma, Literature revien