肺淋巴管肌瘤病并肺母细胞瘤1例临床分析并文献复习  

A case report and review of literature of a patient with pulmonary lymphangiomyomatosis and pneumoblastoma

作　　者：刘杰[1] 陈荣昌[1] 钟南山[1] 李时悦[1] 顾莹莹[2] 欧阳铭[1] 曾庆思[3] 

机构地区：[1]广州呼吸疾病研究所呼吸疾病国家重点实验室呼吸疾病国家临床医学研究中心广州医科大学附属第一医院呼吸内科,510120 [2]广州呼吸疾病研究所病理科,510120 [3]广州呼吸疾病研究所放射科,510120

出　　处：《国际呼吸杂志》2014年第18期1361-1366,共6页International Journal of Respiration

基　　金：“十二五国家科技支撑计划课题（2013BA109809）

摘　　要：目的 加强肺淋巴管肌瘤病（LAM）合并恶性肿瘤的临床、影像、病理特点的认识,提高对该病的早期认识和治疗水平.方法 分析2013年3月收治的1例肺LAM并肺母细胞瘤患者临床资料及诊治经过,并结合相关文献进行复习.截止至2014年2月,以“Lymphangiomyomatosis、cancer”为检索词,在PubMed检索系统进行检索,论著3篇,综述1篇;在万方数据库中以“淋巴管肌瘤病、恶性肿瘤”为检索词进行检索,病例报告1篇.结果 患者,女,37岁,慢性病程,主要症状为咳嗽、咯血、胸痛3个月,既往有“双肾错构瘤”史,曾行右肾错构瘤切除术,术后病理示血管平滑肌脂肪瘤,左肾未予处理.曾有“自发性气胸”病史.胸部高分辨率CT示右中下肺巨大肿块并纵隔淋巴结肿大,考虑恶性病变;肺LAM.血清血管内皮生长因子D为1 092.61 ng/L.免疫组化示CD56灶（＋）,Vim小细胞（＋）,Syn（＋）,CD99（＋/-）,组织改变考虑为肺母细胞瘤.复习国内外文献,尚无LAM合并肺母细胞瘤的病例报道.结论 近十年来,随着LAM分子发病机制的深入研究,LAM目前可定义为低度恶性转移性肿瘤.本例患者为LAM合并高度恶性肿瘤肺母细胞瘤,在国内外尚属首例报道.但究其机制,是LAM细胞癌变为肺母细胞瘤,或是二者同时存在,目前尚无定论.Objective To improve the recognition of clinical features,image,and pathology of pulmonary lymphangiomyomatosis （LAM） and pneumoblastoma and enhance treatment at early stage.Methods A patient with pulmonary LAM and pneumoblastoma was analyzed including clinical data,image and pathological features,and responses of treatment,who was admited in 2013 March.Three articles and one review were searched from January 1967 to February 2014 in PubMed database with ＆quot;lymphangiomyomatosis,cancer＆quot; as the search terms.A case report was searched from January 1982 to February 2014 in Wanfang database.Results The main symptoms of this patient who was female and 37 years old were cough,hemoptysis,and chest pain for three months.Though she had hamartomas of bilateral kidney,she had ectomy of hamartoma of right kidney,and biopsy indicated angiomyolipoma.Spontaneous pneumothorax was occured in past.Chest high-resolution CT indicated right lung malignant disease and pulmonary LAM.The serum vascular endothelial growth factor-D concentration of this patient was 1092.61 ng/L.Lung biopsy confirmed as pneumoblastoma：immunohistochemisty CD56 （＋）,Vim （＋）,Syn（＋）,CD99（＋/-）.Through the review of literature,there was no case of LAM combined with pneumoblastoma reported.Conclusions In recent decade,LAM is defined as a low-grade,destructive,metastasizing neoplasm with the molecule pathogenetic development of LAM.The patient with LAM and pneumoblastoma was first reported in domestic and international literature.However,it is controversial and unclear whether it belongs to carcinomatous change of LAM cell or it originally includes two malignant neoplasms.

关 键 词：淋巴管肌瘤病 肺母细胞瘤 

分 类 号：R734.2[医药卫生—肿瘤]