肺上皮样血管内皮细胞瘤并双侧胸腔积液1例及文献复习

Pulmonary epithelioid hemangioendothelioma with bilateral pleural effusion:a case report and review of the literature

目的加强对肺上皮样血管内皮细胞瘤的认识,提高诊断率。方法通过对1例肺上皮样血管内皮瘤患者的诊治和文献复习,详细分析该病的病因、临床表现、诊断、鉴别诊断、治疗及预后等。结果该病病因不清,临床症状较少且轻微,影像学检查主要表现为两肺多发性结节影,可累及胸膜并出现胸腔积液;病理学检查主要特征是单细胞原始管腔结构;免疫组织化学提示内皮细胞源性。目前尚无有效治疗措施,患者预后介于良、恶性肿瘤之间。结论肺上皮样血管内皮瘤并双侧胸腔积液罕见,极易误诊,当肺部疾病治疗效果不佳时应警惕其可能性。

Objective To understand further and improve the diagnostic accuracy of pulmonary epithelioid hemangioendothelioma. Methods We analyzed the etiology, clinical manifestations, diagnosis, differential diagnosis, management and prognosis of pulmonary epithelioid hemangioendothelioma after diagnosing one case and reviewing some literatures. Results The etiology remained unknown,and symptoms were scanty and usually mild. Chest X-ray or computed tomography usually revealed multiple nodules in bilateral pulmonary. Pleural effusions emerged due to pleura was invaded. Primitive lumen formed by a single cell was the pathological feature. Immunohistochemical stains showed that the cells were original from the endothelial type. There is no effective treatment for this disease and its prognosis is in between benign lession and malignant neoplasm. Conclusion Pulmonary epithelioid hemangioendothelioma with bilateral pleural effusion is rare and often misdiagnosed as other pulmonary diseases.