Daghfous H, Belloumi N, Braham E, Ben Saad S, El Bay R, El Mezni F, Tritar F
Service de pneumologie pavillon C, hôpital Abderrahmen Mami, Ariana 2080, Tunisie.
Service d'anatomopathologie, hôpital Abderrahmen Mami, Ariana 2080, Tunisie.
Rev Pneumol Clin. 2014 Oct;70(5):302-6. doi: 10.1016/j.pneumo.2014.01.001. Epub 2014 Mar 16.
Pulmonary blastoma, a rare primary lung malignancy is subdivided in 3 categories: well-differentiated fetal adenocarcinoma (WDFA), classic biphasic pulmonary blastoma (CBPB) and pleuropulmonary blastoma (PPB). Classic pulmonary blastoma is composed of a mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue.
We described a case of a 48-year-old male, cigarette smoker, who presented with left thoracic pain and hemoptysis for 2 months. Chest radiography showed a well-delimited, homogeneous 4cm mass in the left lung periphery. Bronchoscopic examination revealed left endobronchial bleeding. Computed tomography of the chest revealed a tumor shadow measuring 7cm in the left upper lobe and bilateral nodules with no lymphadenopathy. A systemic evaluation demonstrated no metastatic lesion. Patient underwent a left upper lobectomy. The diagnosis of CBPB was affirmed on anatomopathology of the tumor resection. Immunohistochemical studies showed that tumor cells were positive for vimentin, desmin, actin, Pan Cytokeratin and TTF-1. The final diagnosis was BPB classified as pathological T3N0M0 and no adjuvant treatment was associated. The patient showed good objective response with no evidence of disease recurrence still in 5 years surgery resection.
This case reiterates the importance of pathomorphological or immunohistochemical features in diagnosis of BPB.
肺母细胞瘤是一种罕见的原发性肺恶性肿瘤,分为3类:高分化胎儿腺癌(WDFA)、经典双相性肺母细胞瘤(CBPB)和胸膜肺母细胞瘤(PPB)。经典肺母细胞瘤由类似于胎儿肺组织的未成熟上皮和间充质组织混合组成。
我们描述了一例48岁男性吸烟者,出现左胸痛和咯血2个月。胸部X线显示左肺周边有一个边界清晰、均匀的4cm肿块。支气管镜检查发现左支气管内出血。胸部计算机断层扫描显示左上叶有一个7cm的肿瘤阴影,双侧有结节,无淋巴结肿大。全身评估未发现转移病灶。患者接受了左上叶切除术。肿瘤切除的解剖病理学证实为CBPB。免疫组织化学研究显示肿瘤细胞波形蛋白、结蛋白、肌动蛋白、泛细胞角蛋白和甲状腺转录因子-1呈阳性。最终诊断为BPB,病理分期为T3N0M0,未进行辅助治疗。患者显示出良好的客观反应,术后5年仍无疾病复发迹象。
该病例重申了病理形态学或免疫组织化学特征在BPB诊断中的重要性。