Bini A, Ansaloni L, Grani G, Grazia M, Pagani D, Stella F, Bazzocchi R
Department of Surgical and Anaesthesiological Sciences, Section of General and Thoracic Surgery, University of Bologna, Sant'Orsola-Malpighi Hospital, Via Massarenti 9, 40138 Bologna, Italy.
Surg Today. 2001;31(5):438-42. doi: 10.1007/s005950170136.
Pulmonary blastomas are a group of rare malignant neoplasms subdivided into three categories: classic biphasic pulmonary blastoma (CBPB), well-differentiated fetal adenocarcinoma (WDFA), and pleuropulmonary blastoma (PPB). We report herein the cases of two men with CBPB. Both were heavy smokers and presented with a history of hemoptysis. Physical examination revealed slightly significant findings, chest radiographs showed a large pulmonary mass, confirmed by computed tomography, and bronchoscopic biopsies were not diagnostic. A left and right inferior lobectomy was performed and a diagnosis of CPBP was confirmed by histological examination. In the first patient, local recurrence with multiple bilateral lung metastases was found 6 months later and despite chemotherapy, he died of respiratory failure 1 year after his operation. In the second patient, a subcutaneous metastasis was found in the right subscapular region 2 months later, and a cerebral metastasis in the right posterior parietal lobe 4 months later. Partial remission was achieved by cerebral irradiation, but 6 months later the patient died of cardiac failure while in a coma. We conclude that more aggressive and multidisciplinary treatment should be adopted for CBPB, and because of its low incidence, it is important to unify individual experiences in a central registry to gather as much information as possible regarding the biological and clinical features of this unusual disease.
肺母细胞瘤是一组罕见的恶性肿瘤,分为三类:经典双相性肺母细胞瘤(CBPB)、高分化胎儿腺癌(WDFA)和胸膜肺母细胞瘤(PPB)。我们在此报告两例CBPB男性病例。两人均为重度吸烟者,均有咯血病史。体格检查发现轻微异常,胸部X线片显示肺部有一个大肿块,计算机断层扫描予以证实,支气管镜活检未明确诊断。施行左右下肺叶切除术,组织学检查确诊为CPBP。第一例患者在6个月后出现局部复发并伴有双侧肺部多发转移,尽管进行了化疗,但术后1年死于呼吸衰竭。第二例患者在2个月后右侧肩胛下区域发现皮下转移,4个月后右侧顶叶后部发现脑转移。脑部放疗后达到部分缓解,但6个月后患者在昏迷中死于心力衰竭。我们得出结论,对于CBPB应采用更积极的多学科治疗,并且由于其发病率低,在中央登记处统一个人经验以收集关于这种罕见疾病的生物学和临床特征的尽可能多的信息非常重要。