以咳血丝痰及反复气胸为主要表现的肺上皮样血管内皮瘤1例并文献复习  

Pulmonary epithelioid hemangioendothelioma with main clinical manifestations as blood-tinged-sputum-cough and pneumothorax：one case report and literature review

作　　者：张筱娴[1] 刘春丽[1] 王璐琳[1] 周圆明[1] 李时悦[1] 张挪富[1] 

机构地区：[1]广州医科大学附属第一医院广州呼吸疾病研究所呼吸疾病国家重点实验室,510120

出　　处：《国际呼吸杂志》2015年第6期427-431,共5页International Journal of Respiration

摘　　要：目的总结肺上皮样血管内皮瘤的特征，以提高临床医师对该病的认识。方法通过对1例肺上皮样血管内皮瘤病例的临床、影像学及病理分析，并复习肺上皮样血管内皮瘤的相关文献，从而总结其特征。结果首次报道以双肺反复出现气胸为临床表现及双肺逐步出现多个小囊状病灶为影像学特点的肺上皮样血管内皮瘤。结论对于咳血丝痰、双肺反复出现气胸、影像学表现为双肺多个小结节、散在渗出病变及双肺逐步出现多个小囊状病灶的患者需考虑肺上皮样血管内皮瘤，最终确诊有赖于组织病理学及免疫组化检查。Objective To summarize the characteristics of pulmonary epithelioid hemangioendothelioma and improve knowledge about the disease for physicians. Methods The clinical, radiological and pathological characteristics of one patient with pulmonary epithelioid hemangioendothelioma were analyzed and the related literatures were reviewed. Results The clinical manifestation of recurrent pneumothorax in bilateral lungs and the imaging finding of gradually appearance of cystic lesions in bilateral lungs for pulmonary epithelioid hemangioendothelioma were first reported in this paper. Conclusions Pulmonary epithelioid hemangioendothelioma should be considered when patients present with blood-tinged-sputum-cough, recurrent pneumothorax in bilateral lungs, imaging findings of multiple nodules,diffused exudative lesions and gradually appearance of cystic lesions in bilateral lungs. The final diagnosis depends on histopathological and immunohistochemical examination.

关 键 词：肺上皮样血管内皮瘤 咳血丝痰 气胸 小囊状病灶 

分 类 号：R734.2[医药卫生—肿瘤]