20例成人肺母细胞瘤临床分析

Pulmonary blastoma in adults: A clinical analysis of 20 cases

  • 摘要: 目的 分析成人型肺母细胞瘤的临床特点、诊断及治疗。 方法 回顾性总结1993年1月-2012年9月我科收治的肺母细胞瘤20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 17例行手术治疗,3例行保守治疗。其中肺叶切除15例,全肺切除2例。3例保守治疗者经穿刺病理确诊,17例手术病例术前均未确诊。免疫组化示4例双相型间叶成分Vimentin(+),3例原始上皮成分AE1/AE3(+),3例SMA(+),2例CgA(+)。平均随访21月,平均生存时间14.8月。 结论 成人型肺母细胞瘤临床少见,症状无特征性,术前确诊困难,联合应用免疫组化标记有助于鉴别诊断;手术是治疗的第一选择,但易复发和转移,预后不佳。

     

    Abstract: Objective To analyze the clinical features, diagnosis and treatment of pulmonary blastoma (PB) in adults. Methods Clinical manifestations, pathological characteristics, diagnosis and treatment of 20 PB patients admitted to our hospital from January 1993 to September 2012 were retrospectively analyzed with its relative literature reviewed. Results Of the 20 cases, 17 with their diagnosis not established underwent surgical treatment (15 underwent lobectomy and 2 underwent pneumonectomy) and 3 diagnosed by fineneedle pulmonary aspiration received conservative treatment. Immunohistochemistry showed positive vimentin in 4 biphasic cases, positive AE1/AE3 in 3 cases, positive SMA in 3 cases, and positive CgA in 2 cases. The patients were followed up for an average period of 21 months. The median survival time of the patients was 14.8 months. Conclusion PB is rare in adults with no typical symptoms and is thus difficult to establish its diagnosis before operation. Immunohistochemistry marker can help its differential diagnosis. Surgery is the first choice of treatment. Relapse and metastasis of PB are easy to occur and its prognosis is poor.

     

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