摘要
目的分析总结儿童I型胸膜肺母细胞瘤(pleuropulmonary blastoma,PPB)的临床特征及影像表现,提高对本病的认识,减少误诊。方法回顾性分析经手术病理证实的9例儿童I型PPB的临床及影像学资料。所有儿童均行胸部X线摄影及CT平扫+增强检查。结果9例均表现为上呼吸道感染症状,2例合并其他疾病,1例有家族史。术前X线阳性率100%,但特异性低。CT诊断符合率为22.2%,余均误诊为先天性肺气道畸形(congenital pulmonary airway malformations,CPAM)。病灶4例发生于右肺,5例发生于左肺。9例均表现为巨大多房囊性肿物,囊肿最大径5.2~12.7 cm,平均直径(9.2±2.9)cm。囊壁结节2例,囊壁钙化1例,间隔增厚9例。增强扫描间隔强化2例、结节强化1例。6例合并感染和纵隔移位,1例合并气胸。结论I型PPB是儿童最常见肺部囊性恶性肿瘤,好发于周围肺泡或胸膜组织,呈巨大多房囊性肿物,容易合并肺炎、纵膈移位及气胸。囊壁增厚、钙化及结节形成是CT诊断的重要依据。胸部CT是诊断儿童I型PPB首选检查方法。
Objective To summarize and analyze the clinical and imaging features of type I pleuropulmonary blastoma(PPB)in children,and to improve the understanding of the disease so as to reduce misdiagnosis.Methods The clinical manifestations and imaging findings of 9 children of type I PPB proved by pathologically were analyzed retrospectively.All 9 cases underwent chest X-ray and chest plain and contrast CT examinations.Results All 9 children had no history of prenatal diagnosis and showed symptoms of upper respiratory tract infection.2 children had combined malformations and 1 had a family history.The positive rate of X-ray was 100%,but the specificity was low.The accuracy of CT diagnosis preoperative was 22.2%,and the rest were misdiagnosed as congenital pulmonary airway malformations(CPAMs).4 lesions occurred in the right lung and 5 lesions occurred in the left lung.9 cases showed huge multilocular cystic masses.The maximum diameter of the cyst was from 5.2 cm to 12.7 cm,with means about(9.2±2.9)cm.Nodules of the cystic wall were found in 2 cases,calcification of the cystic wall in 1 case,and the thickened interval in 9 cases.2 cases with intervals and 1 case with nodules were enhancement.6 cases showed pneumonia and mediastinal shift,and 1 case showed pneumothorax.Conclusion Type I PPB was the most common cystic malignancy lung tumor in children and occurred in alveolar or pleural tissues around the chest.It presented a huge multi-cystic mass with pneumonia,mediastinal shift and pneumothorax.Thickening of the cyst wall,calcification,and nodule formation were the important features in CT diagnosis.Chest CT was the preferred method of diagnosis for type I PPB in children.
作者
田金生
吴慧莹
周成
刘强
刘鸿圣
徐文彪
鹿连伟
李建明
TIAN Jinsheng;WU Huiying;ZHOU Cheng;LIU Qiang;LIU Hongsheng;XU Wenbiao;LU Lianwei;LI Jianming(Department of Imaging Center,Guangzhou Women and Children’s Medical Center,Guangzhou 510120,P.R.China;Department of Radiology,Guangzhou University of Chinese Medicine Affiliated Clifford Hospital,Guangzhou 511495,P.R.China)
出处
《医学影像学杂志》
2021年第7期1155-1159,共5页
Journal of Medical Imaging
