肺母细胞瘤的临床分析及文献复习

Clinical analysis and review of literature of pulmonary blastoma

目的分析肺母细胞瘤的临床特点、诊断及治疗方法。方法回顾性地总结自1990年2月至2005年10月我科收住的肺母细胞瘤4例，并结合文献对其临床表现、病理特征、诊断及治疗方法进行分析。结果肺母细胞瘤由原始的上皮成分和原始的间叶成分共同组成。4例肺母细胞瘤主要症状为咳嗽、血丝痰等，X线及CT均表现为周围型、单发、病灶密度不均、边缘清楚，病灶大小在3—7cm。本组4例术前均未确诊，均为术后病理确诊。3例患者术后1至2年出现转移或复发。3例随访至今，2例仍生存（分别为4年和2年），1例术后生存2年。结论肺母细胞瘤是一种罕见的含有原始上皮成分和原始间叶成分的双向性肿瘤，主要见于成人，术前确诊困难，预后差，外科手术是其主要的诊断和治疗手段。

Objective To study the clinical characteristics and diagnosis, treatment of pulmonary blastoma（PB）. Methods Four cases of PB from February 1990 to October 2005 were summarized retro- spectively, and literature was reviewed to analyse their clinical manifestation, pathologic features, diagnosis and treatment. Results PB of the 4 cases were composed of primitive epithelial component and primitive stromal component. The main symptoms of these four cases PB were cough, bloody spatum. X-ray or CT scan showed an isolated mass in peripheral area of lung, with in homogeneous density and clear margin. The mass size in from 3 to 7 cm. The four cases were not diagnosed definitely before operation and were diagnosed after operation. Three patients were found to have metastases or recurrence 1 to 2 years after surgical treatment. Three cases were followed up, of them, 2 are still alive（4 years and 2 years, respectively） and 1 case survived for 2 years. Conclusions PB is a rarely seen biphasic tumor which contains primitive epithelial component and primitive mesenchymal stroma. It is mainly found in adult. It is very difficult to reach a definite diagnosis preoperatively and prognosis is very poor. Surgical resection is the main method of diagnosis and treatment.