Mlika Mouna, Anjum Fatima, El Mezni Faouzi
University of Medicine of Tunis
Temple University, Lewis Katz School of Medicine
Childhood tumors of the lung are rare malignant tumors accounting for 0.5% to 1% of all primary malignant lung tumors. These tumors are subdivided into 3 subtypes: pulmonary blastoma, fetal adenocarcinoma, and pleuropulmonary blastoma (PPB). An epithelial malignant and immature component characterizes fetal adenocarcinoma. A malignant immature mesenchymal proliferation characterizes the pleuropulmonary blastoma. Biphasic components define the blastoma; a mesenchymal and an epithelial malignant and immature component, that look like a 10- to 16-week gestational lung. In the 2015 World Health Organization Classification, fetal adenocarcinoma belongs to the group of adenocarcinomas, pulmonary blastoma belongs to the group of sarcomatoid carcinoma, and pleuropulmonary blastoma belongs to the group of mesenchymal tumors. Pleuropulmonary blastomas are rare tumors which present non-specific symptoms. Although the importance of the radiologic features to suspect the diagnosis, the positive diagnosis remains based on the microscopic features. Many synonyms of pleuropulmonary tumors exist in the literature including: Cystic mesenchymal hamartoma. Mesenchymal cystic hamartoma. Pediatric pulmonary blastoma. Pneumoblastoma. Pulmonary rhabdomyosarcoma. Rhabdomyosarcoma in lung cyst.
儿童肺部肿瘤是罕见的恶性肿瘤，占所有原发性恶性肺部肿瘤的0.5%至1%。这些肿瘤可细分为3种亚型：肺母细胞瘤、胎儿型腺癌和胸膜肺母细胞瘤（PPB）。胎儿型腺癌的特征是上皮性恶性和不成熟成分。胸膜肺母细胞瘤的特征是恶性不成熟间叶组织增生。双相成分定义了母细胞瘤；一个间叶组织和一个上皮性恶性和不成熟成分，看起来像妊娠10至16周的肺组织。在2015年世界卫生组织分类中，胎儿型腺癌属于腺癌组，肺母细胞瘤属于肉瘤样癌组，胸膜肺母细胞瘤属于间叶组织肿瘤组。胸膜肺母细胞瘤是罕见的肿瘤，表现为非特异性症状。尽管放射学特征对怀疑诊断很重要，但确诊仍基于微观特征。文献中存在许多胸膜肺肿瘤的同义词，包括：囊性间叶组织错构瘤、间叶组织囊性错构瘤、儿童肺母细胞瘤、肺母细胞瘤、肺横纹肌肉瘤、肺囊肿内横纹肌肉瘤。
