摘要
目的:探讨非典型年龄(青春期及成人)先天性囊性腺瘤样畸形(CCAM)的临床病理学特征。方法:回顾性分析2019年1月—2024年12月中日友好医院确诊的36例青春期及成人CCAM病例,收集患者临床表现、影像学特征、病理分型及免疫组化结果,结合文献进行对比分析。结果:患者年龄17~65岁,中位年龄38岁;男14例(39%),女22例(61%)。病变以左肺下叶与右肺下叶最常见(各占38.9%)。临床多表现为体检发现肺部占位或慢性咳嗽。病理类型包括1型23例(58.3%)、2型9例(27.8%)、3型4例(13.9%)。31例(86.1%)可见不同程度胸膜改变,20例(55.6%)出现大小不一囊腔,15例(41.7%)伴炎细胞浸润,另有少数病例可见坏死、钙化、纤维化等改变。1例(2.8%)合并黏液腺癌。免疫组化显示TTF-1、NapsinA阳性,P63、P40、CK5/6在基底细胞层表达明确。随访发现仅1例术后2年复发。结论:青春期及成人发病的CCAM属于非典型年龄发病类型,临床表现隐匿,易被误诊为其他肺部肿瘤。其病理形态和免疫表型特征明确,诊断价值高。
Objective:To explore the clinical presentations,imaging features,pathological subtypes,and immunohistochemical profiles of patients with congenital cystic adenomatoid malformation(CCAM)in adolescents and adults.Methods:A retrospective analysis was conducted on 36 cases of CCAM diagnosed at the Department of Pathology,China-Japan Friendship Hospital,between Jan 2019 and Dec 2024.All cases occurred in atypicalage groups(adolescence or adulthood).Clinical data,pathological observations,and immunohistochemical(IHC)findings were reviewed alongside relevant literature.Results:The cohort included 14 males(39%)and 22 females(61%),with a median age of 38 years.Lesions were most frequently located in the left and right lower lobes(each 38.9%).Clinically,most cases were identified incidentally during routine examination or presented with chronic cough.Pathological subtypes included type 1(23 cases,58.3%),type 2(9 cases,27.8%),and type 3(4 cases,13.9%).Pleural involvement was noted in 31 cases(86.1%),cystic structures of varying sizes in 20 cases(55.6%),and inflammatory infiltration in 15 cases(41.7%).Necrosis,calcification,or fibrosis were observed in a few patients.One case(2.8%)was associated with mucinous adenocarcinoma.Immunohistochemistry showed TTF-1 and NapsinA positivity in the epithelium,with P63,P40,and CK5/6 positivity in the basal cell layer.During follow-up,only one patient experienced recurrence two years postoperatively.Conclusion:CCAM in adolescents and adults represents an atypical-age variant of this congenital pulmonary malformation.It presents with subtle clinical signs and is often misdiagnosed as other pulmonary cystic or neoplastic lesions.Characteristic histological features and immunoprofiles support a benign developmental anomaly.
作者
伍景鹏
支星淇
孙培宇
钟定荣
WU Jing-peng;ZHI Xing-qi;SUN Pei-yu(China-Japan Friendship Hospital(Institute of Clinical Medical Sciences),Chinese Academy of Medical Sciences&Peking Union Medical College,Beijing 100029,China)
出处
《中日友好医院学报》
2025年第4期226-229,F0004,共5页
Journal of China-Japan Friendship Hospital
基金
国家自然科学基金(82473138)
中央高水平医院临床科研业务费(2022-NHLHCRF-LX-01-0206)
中国医学科学院医学科学创新基金(2023-I2M-C&T-B-120)。
关键词
先天性囊性腺瘤样畸形
青春期
成人
肺疾病
免疫组化
congenital cystic adenomatoid malformation
adolescents
adults
lung disease
immunohistochemistry
作者简介
伍景鹏(2000一),男,住院医师,硕士研究生;通信作者:钟定荣。
