成人型肺母细胞瘤五例报告及文献复习

Pulmonary blastoma: a report of five cases and review of the literature

背景与目的肺母细胞瘤是一种少见的肺部原发恶性肿瘤,分为成人型肺母细胞瘤和儿童胸膜肺母细胞瘤.本文主要讨论成人型肺母细胞瘤的临床特点、诊断和治疗.方法回顾我院从1964年4月至2004年3月收治的5例成人型肺母细胞瘤的临床资料,并复习有关国内外文献.结果 5例中男3例,女2例;发病年龄22～70岁;以咳嗽、咳血丝痰为主要症状,次为胸闷、胸痛;影像学主要表现为肺部单发实性肿块,纤维支气管镜检查及痰细胞学检查均没有发现肿瘤细胞.3例行肺叶切除加肺门纵隔淋巴结清扫术,1例行左全肺切除加肺门纵隔淋巴结清扫术,1例行肺楔形切除术并行术后化疗.到随访结束,3例死亡,2例生存,生存期最短6个月,最长11年.结论肺母细胞瘤术前难明确诊断.治疗以肺叶切除加纵隔淋巴结清扫术为主,对放疗及化疗不敏感,预后差别较大.

Background and objective Pulmonary blastoma is a rare primary malignancy of the lung. It is now recognized in two forms: adult type pulmonary blastoma and childhood pleuropulmonary blastoma. The clinical characteristics, diagnosis and treatments of adult type pulmonary blastoma are discussed in this article. Methods The clinical records of 5 patients with adult type pulmonary blastoma admitted in Cancer Center, Sun Yet-sen University from 1964 to 2004 were analyzed and the literature on pulmonary blastoma was reviewed. Results Three patients were male and two were female with the ages ranged from 22 years old to 70. Their symptoms consisted mainly of cough, hemoptysis and chest pain. The pulmonary blastomas were mainly manifested as a solitary parenchymal mass of the lung on chest radiograph and CT. None of these patients was diognosed by fibrobronchoscopy nor sputum cytology. Three patients underwent lobectomy, one underwent pneumonectomy, and these four patients underwent mediastinal lymph node resection also. The fifth one received wedge resection and postoperative chemotherapy. At the end of follow-up, three patients died and two was alive, and the survival time was from 6 months to 11 years. Conclusion Pulmonary blastoma is difficult to be diagnosed before operation. Surgery is the best therapeutic choice up to now. It has poor response to radiotherapy and chemotherapy. The prognosis of patient with pulmonary blastoma is variable.