儿童胸膜肺母细胞瘤的影像学分析

Imaging Analysis of Pleuropulmonary Blastoma in Children

目的:探探讨胸膜肺母细胞瘤的影像学特征,提高对本病的认识。方法:回顾性分析21例(上海市儿童医院13例,上海市胸科医院8例)于2014年6月-2016年4月间经手术病理证实的胸膜肺母细胞瘤的CT和MRI的影像学表现。结果:21例中囊实性15例,实性6例,未见单纯囊性。CT表现:胸膜下肺外带囊实性或实性巨大软组织肿块,胸膜下脂肪线消失;病灶广泛累及胸膜,可侵犯邻近组织,出现胸腔积液、胸壁软组织包块;增强扫描肿瘤实性成分呈不均匀强化,囊性成分无强化,部分有延迟强化。MRI表现:T1WI呈等低信号,T2-FS呈混杂等高信号,增强呈不均强化。MRI对肿块范围显示更具优势。结论:影像学表现对胸膜肺母细胞瘤的诊断具有较高的提示意义,尤其在CT表现为胸膜下肺外带的巨大软组织肿块及胸壁广泛侵犯、MRI表现为更大范围的混杂信号软组织肿块时,是诊断胸膜肺母细胞瘤重要的影像学依据,确诊需靠病理及免疫组化查证。

Purpose：To study the imaging characteristics of pulmonary blastoma （PB）, and to improve the acknowledgement of this disease. Methods：CT and MRI imaging data from 21 cases of PB in Children＇s hospital of Shanghai （13 cases） and Shanghai chest hospital （8 cases）, from June 2014 to April 2016, were retrospectively analyzed. Results：21 cases, fifteen lesions were cystic-solid, six were solid. CT films demonstrated huge tumors in thoracic cavity which extruded the lungs or mediastinum, and adjacent extrapleural fat line disappeared. Pleura were extensively involved. Adjacent tissues were invaded, while pleural effusion and chest wall tumors could be found on some patients. After injection of contrast agent, solid components enhanced heterogeneously. Delay enhancement could be found on some lesions. MRI findings： Equal or low signal on T1W imaging, high signal combined with equal signal on T2W imaging and heterogeneous enhancement could be observed. MRI demonstrates the margin better than CT. Conclusion：Thare are some imaging characteristics of pulmonary blastoma. In paticular, large subpleural complex signal tumor on MRI imaging and widespread pleural invasion on CT imaging are helpful to diagnosis. Pathology and immunohistochemistry should be taken to confirm it.