Liu Yi, Luo Dongbo, Du Tongxin, Wang Hongjiang
Department of Thoracic Surgery, Xinjiang Medical University Affiliated Tumor Hospital, Urumqi, Xinjiang.
Medicine (Baltimore). 2017 Dec;96(50):e8918. doi: 10.1097/MD.0000000000008918.
Pulmonary blastoma is a rare primary lung cancer that can be categorized into adult type and child type. The clinical symptoms and imaging features of pulmonary blastoma are nonspecific, making it difficult to diagnose preoperatively. Postoperative pathology with immunohistochemical staining can help diagnosis.
A 53-year-old male had chest tightness and shortness of breath.
The patient was diagnosed as pleural pulmonary blastoma based on computed tomography (CT) scan, pathology, immunohistochemistry, and molecular pathology. CT examination showed solid mass on the upper lobe of the left lung Intraoperative observation found that tumor tissue was gray with tough texture. The surrounding lung tissue showed AE1/AE3 (+), Vimentin (+), and CD34 (+) staining. No epidermal growth factor receptor gene mutation was detected.
The left lobe resection plus mediastinal lymph node dissection were performed. After the operation, patient received paclitaxel combined with nedaplatin chemotherapy for 4 times.
Four months later, left pleural metastasis, and mediastinal lymph node metastasis was found. The patient died 15 months later.
Pleural pulmonary blastoma is a malignant tumor with rare pathological features that is easy to relapse and metastasis with poor prognosis. Surgical treatment preferably, lobectomy plus mediastinal lymph node dissection, is the first treatment option. The overall prognosis is poor.
肺母细胞瘤是一种罕见的原发性肺癌，可分为成人型和儿童型。肺母细胞瘤的临床症状和影像学特征不具有特异性，术前难以诊断。术后病理检查及免疫组化有助于诊断。
一名53岁男性，有胸闷、气短症状。
根据计算机断层扫描（CT）、病理、免疫组化及分子病理学检查，患者被诊断为胸膜肺母细胞瘤。CT检查显示左肺上叶实性肿块。术中观察发现肿瘤组织呈灰白色，质地坚韧。周围肺组织AE1/AE3（+）、波形蛋白（+）、CD34（+）染色阳性。未检测到表皮生长因子受体基因突变。
行左肺叶切除加纵隔淋巴结清扫术。术后患者接受紫杉醇联合奈达铂化疗4个周期。
4个月后，发现左侧胸膜转移及纵隔淋巴结转移。患者于15个月后死亡。
胸膜肺母细胞瘤是一种具有罕见病理特征的恶性肿瘤，易复发和转移，预后较差。首选手术治疗，即肺叶切除加纵隔淋巴结清扫，是首选的治疗方案，但总体预后不佳。
