叶内型肺隔离症的诊断、治疗及分子病理学特点  

Diagnosis and treatment of intralobar pulmonary sequestration

作　　者：吴楠[1] 孙宇[2] 郑庆锋[1] 吕超[1] 阎石[1] 张力建[1] 杨跃[1] 

机构地区：[1]北京大学临床肿瘤学院北京肿瘤医院暨北京市肿瘤防治研究所胸外科,100036 [2]北京大学临床肿瘤学院北京肿瘤医院暨北京市肿瘤防治研究所病理科,100036

出　　处：《中华医学杂志》2007年第37期2627-2631,共5页National Medical Journal of China

基　　金：国家自然科学基金(30470853)

摘　　要：目的总结叶内型肺隔离症的临床病理学特点,并了解在慢性炎症状态下肿瘤相关蛋白 p53和癌胚抗原(CEA)的表达。方法收集并分析7例顺内型肺隔离症的临床特征、手术治疗模式及病理学特点,并应用免疫组织化学染色检测肺隔离症组织 p53和 CEA 的表达。结果本组7例叶内型肺隔离症主要症状为肺部感染。患者均在术前确诊并接受手术治疗,主要为叶切除术。异常体循环血管均发白胸主动脉。病变肺组织呈不同程度的囊性扩张,部分上皮增生。6例囊肿上皮全部呈 p53蛋白阳性表达,定位于黏膜上皮的顶层。CEA 在6例上皮中呈弥漫性表达。而叶支气管断端黏膜上皮 p53和 CEA 均表达阴性。结论叶内型肺隔离症可通过增强 CT 并三维重建技术确诊,多采用叶切除术治疗。肺隔离症的囊肿上皮,特别是增生上皮的顶层可以出现 p53蛋白异常积聚和CEA 的表达,可能与局部慢性炎性刺激有关。Objective To summarize the diagnosis and surgical treatment of intralobar pulmonary sequestration （PS）. Methods The clinical data of 7 cases of intralobar PS, 5 males and 2 females, aged 15 -38, was collected and analyzed. Macroscopic and microscopic pathological findings were recorded. The expression of protein p53 and carcinoembryonic antigen （CEA） was evaluated immunohistochemically in 6 samples obtained from lobectomy. Results All 7 patients were admitted with major features of intermittent lung infection. Diagnosis was confirmed in all 7 cases before operation by contrast enhanced helical CT or MRI, All patients were treated with surgical excision, of which lobectomy was performed in 6 cases and wedge resection in 1 case. No surgical death was reported. All the aberrant systemic arteries arose from the thoracic aorta. The histological pictures showed polycystic lesion in sequestrated area with fibrosis formation and chronic inflammatory cell infiltration in the surrounding pulmonary stroma. Hyperplasia of epithelium occurred in some parts of the cystic lesions. Positive protein p53 staining and diffuse CEA expression were detected in all the 6 cases, showing stronger protein p53 staining in whose superficial layer of hyperplastic epithelium than in thee basal layer. The normal bronchial epithelium was not stained with p53 or CEA. Conclusion The diagnosis of intralobar PS can be confirmed by enhanced contrast helical CT with 3- dimentional reconstruction, a non-invasive method. Surgical intervention, such as lobectomy, can be applied after complete control of pulmonary infection. Aberrant accumulation of p53 protein and CEA expression in the cystic epithelium inside PS tissues show a relationship with chronic inflammation.

关 键 词：支气管肺隔离症 诊断 治疗 蛋白质P53 痛胚抗原 

分 类 号：R655.3[医药卫生—外科学]