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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 investigate the clinicopathological features of congenital cystic adenomatoid deformity (CCAM). Methods Four cases of CCAM confirmed by surgery and pathology were analyzed clinically and pathologically. The literature was reviewed systematically to discuss the pathological classification, clinical features, treatment and prognosis and their pathogenesis. Results 4 cases were involved in unilateral unilateral leaf lung, including 3 cases of CCAM Ⅰ, Ⅱ 1 case. In addition to 1 case of fetus abortion, the remaining 3 cases of partial lung resection or lobectomy. Conclusion CCAM is a rare pulmonary congenital malformation, imaging is an effective means to detect the disease, the diagnosis depends on the pathological examination. CCAM patients have a certain risk of malignancy, surgical removal of diseased lung tissue is an effective treatment.