Regragui A, Cherradi N, Sefiani S, Malihy A, Khattab M, Alhamany Z
Service d'Anatomie Pathologique, Hôpital d'Enfants, CHU Ibn Sina, Rabat, Maroc.
Rev Pneumol Clin. 2003 Dec;59(6):365-70.
Pleuropulmonary blastoma is a very exceptional anatomoclinical and histological entity recently distinguished from adult pneumoblastoma. This tumor, observed in children aged less than 15, can involve the lung, the pleura, or the mediastinum and is characterized by a very poor prognosis. We report the case of a 4-year-old girl who developed pleuropulmonary blastoma which was discovered in a context of respiratory distress. Standard chest x-ray revealed an opacity covering the entire left lung. Histology and immunohistology led to the diagnosis of pleuropulmonary blastoma with several components: blastematous, malignant mesenchymatous with pluridirectional differentiation, and benign epithelial tissue. Treatment consisted in preoperative chemotherapy and radiotherapy to reduce tumor volume. This neoadjuvant treatment is not widely reported and its relatively favorable result allowed tumor resection. This approach might be useful in similar cases.
胸膜肺母细胞瘤是一种非常罕见的解剖临床和组织学实体，最近才与成人肺母细胞瘤区分开来。这种肿瘤见于15岁以下儿童，可累及肺、胸膜或纵隔，预后极差。我们报告一例4岁女孩发生胸膜肺母细胞瘤，该病例是在呼吸窘迫情况下被发现的。标准胸部X线显示整个左肺有一片模糊阴影。组织学和免疫组织学检查确诊为胸膜肺母细胞瘤，具有多种成分：胚基性、具有多向分化的恶性间叶性成分以及良性上皮组织。治疗包括术前化疗和放疗以缩小肿瘤体积。这种新辅助治疗方法报道较少，其相对良好的效果使肿瘤得以切除。这种方法可能对类似病例有用。
