肺上皮样血管内皮瘤并发慢性阻塞性肺疾病1例报告及文献复习

Pulmonary epithelioid hemangioendothelioma complicated with chronic obstructive pulmonary disease:A case report and literature review

目的:分析肺上皮样血管内皮瘤(PEHE)并发慢性阻塞性肺疾病(COPD)患者的临床表现、诊断及治疗经过,提高临床医生对PEHE的认识。方法:收集1例并发COPD的PEHE患者临床资料,并结合相关文献,总结其临床诊断和治疗方法。结果:患者,男性,74岁,因反复胸闷、气喘半年,加重半月入院。查体,桶状胸,叩诊两肺过清音,呼吸音减低,无其他明显阳性体征。肺功能,第1秒用力呼气容积(FEV1)占预计值58%,FEV1/用力肺活量(FVC)56.9%。胸部CT检查结果提示两肺多发大小不等结节,两肺慢性支气管炎及肺气肿改变伴肺大泡形成。支气管镜活检见异型细胞巢,结合临床和免疫标记符合PEHE诊断。给予恩度和支气管扩张剂治疗后好转并出院。后续患者胸闷、气喘症状再发,以COPD急性加重入院对症治疗,继续采用恩度抗肿瘤治疗,患者症状改善不明显,并伴有乏力和全身多处骨关节疼痛。影像学评估提示患者出现肝内转移灶和多发骨转移,改用安罗替尼口服抗肿瘤及对症治疗。此后患者病情继续进展,出现呼吸衰竭、低蛋白血症和继发感染,影像学评估两肺病灶进展,对症及支持治疗无效,最终患者死亡。结论:PEHE患者临床症状不典型,并发COPD时更易被忽视,COPD的存在也同样会影响治疗方案的选择。

Objective:To analyze the clinical manifestation,diagnosis and treatment of the patient with pulmonary epithelioid hemangioendothelioma(PEHE)combined with chronic obstructive pulmonary disease(COPD),and to develop the clinicians’understanding of PEHE.Methods:The clinical data of a patient with PEHE complicated with COPD were collected;the relevant literatures were reviewed,and its diagnosis and treatment methods were summarized.Results:The male patient was 74 years old,who was hospitalized because of repeated chest tightness and asthma for half a year and half a month of deterioration.Physical examination showed barrel chest,and hyperresonant sounds were heard when percussing lungs;the respiratory sounds of lungs were reduced;there were no other obvious positive signs.The examination results of pulmonary function showed that forced expiratory volume in 1 second(FEV1)accounted for 58%of predicted value and FEV1/forced vital capacity(FVC)was 56.9%.The chest CT results showed multiple nodules with different sizes,chronic bronchitis and bullous emphysema in both lungs.The bronchoscope results showed abnormal cell-nest,the clinical and immunologic markers were considered,and the results met the diagnosis of PEHE.The patient got better after treated with endostar and bronchodilator and was discharged from hospital.After that,chest tightness and asthma occurred again,so the patient was admitted to the hospital for symptomatic treatment because of COPD,but the symptoms were not relieved significantly and fatigue and systemic pain were found after endostar was given.The evaluation of imaging revealed intrahepatic metastases,and bone metastases in several areas,so the patient received anti-tumor and symptomatic treatment by taking anlotinib.Then,respiratory failure,hypoalbuminemia and secondary infection occurred.So symptomatic treatment was given after assessing the lesions of both lungs by imaging.But the patient was dead.Conclusion:The clinical symptoms of PEHE are atypical,and it is easy to be ignored when a patient is accompanied by COPD.The presence of COPD can also affect what kind of treatment plan can be used.