肺上皮样血管内皮细胞瘤1例并文献复习

Pulmonary epithelioid hemangioendothelioma: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。方法结合广州呼吸疾病研究所2004-05-26收治的1例肺上皮样血管内皮细胞瘤患者的临床资料和文献复习,详细分析该病的病因、临床特点、病理诊断、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,多为咳嗽、咳痰、少量咯血;影像学检查主要表现为两肺多发性结节影;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,预后介于良、恶性肿瘤之间。结论肺上皮样血管内皮细胞瘤罕见,极易误诊,应予重视。

Objective To describe the clinical features of pulmonary epithelioid hemangioendothelioma and heighten the ratio of diagnosis. Methods With a case report and review of the related literature, the etiology, clinical manifestations, pathology diagnosis, differential diagnosis, management and prognosis of pulmonary epithelioid hemangioendothelioma were described. Results The etiology of this rare disease remained unknown. Symptoms were scanty and usually mild. Chest radiograph or computed tomography usually revealed multiple bilateral pulmonary nodules. Primitive lumen formed by a single cell was the pathologic feature. Immunohistochemical stains showed that the malignant cells are of the endothelial type. There was no effective treatment for this disease and its prognosis was unpredictable. Conclusion Pulmonary epithelioid hemangioendothelioma is rare and often misdiagnosed as other pulmonary diseases.