肺先天性囊性腺瘤样畸形4例临床病理分析

Congenital cystic adenomatoid malformation of lung: a clinicopathologic study of 4 cases

目的探讨肺先天性囊性腺瘤样畸形(CCAM)的临床病理特征。方法对4例经手术、病理证实的CCAM病例进行临床病理分析,并系统复习文献,讨论其病理分型、临床特点、治疗和预后及其发病机制。结果4例均累及单侧单叶肺,其中CCAMⅠ型3例,Ⅱ型1例。除1例为胎儿行引产外,其余3例行部分肺切除或肺叶切除术。结论CCAM是一种少见的肺先天性畸形,影像学是发现该病的有效手段,确诊有赖于病理检查。CCAM患者有一定的恶变风险,手术切除病变肺组织是有效的治疗方法。

Objective To study the clinicopathologic features of congenital cystic adenomatoid malformation （CCAM） of lung. Methods The clinical and pathologic findings were evaluated in 4 cases of CCAM confirmed by operation and pathologic analysis. The pathological classification, clinical features, treatment, prognosis and pathogenesis were discussed later with review of the related literature. Results Lesions involved unilateral and single lobe in all 4 patients, including 3 cases of CCAM type Ⅰ , and 1 case of CCAM type Ⅱ . Three cases received segmentectomy or lobectomy, except that one case experienced induced abortion. Conclusion CCAM is a rare congenital malformation of lung. Imaging is the effective method to detect CCAM, while the diagnosis depends on pathological examination. Patients with CCAM are at increased risk of developing malignancy. Resection of lesions from the lung is considered as the most effective treatment.