3例主动脉瘤及主动脉夹层合并主动脉良性肿瘤及瘤样病变的临床病理学分析

目的:发生在主动脉的良性肿瘤并伴有主动脉疾病(主动脉瘤、主动脉夹层及马方综合征)是少见病例。本文总结分析伴有主动脉疾病的主动脉良性肿瘤及瘤样病变的临床病理特点,复习发生于主动脉良性肿瘤的文献,探讨其发生与主动脉疾病之间的关系。方法:回顾性分析我院病理科2006年至2012年,手术切除的胸主动脉瘤及胸主动脉夹层的标本129例,通过复习临床病历,观察HE染色切片,辅以弹力/VG及Masson等组织化学染色及SMA、CD31及CD34等免疫组织化学染色,对主动脉壁结构的改变进行分析。结果:所有病例主动脉壁均发生了结构的改变,主要是中膜弹力纤维、平滑肌及基质的变化,但有3例标本内膜增生明显,1例形成了平滑肌瘤,另2例发生了内膜下弹力纤维瘤样增生及平滑肌瘤样增生。结论:主动脉壁内不同组成成分的改变,使得主动脉壁重构,导致主动脉瘤及主动脉夹层的形成,而某一单一成分过度增生可形成主动脉腔内的肿瘤。

Clinicopathologic analysis of 3 cases of aortic aneurysms and aortic dissections with aortic tumor and tumor-like lesions

Objective： It is rare that aortic neoplasm accompanied with aortic diseases （aortic aneurysm, aortic dissection and Marian syndrome）. To investigate the relationship between aortic tumor and aortic aneurysm, cases of aortic tumors with aortic aneurysms and aortic dissections were analyzed and literatures reviewed. Methods： One hundred and twenty nine thoracic aortic aneurysm（TAA） and thoracic aortic dissection （TAAD） were reviewed at Anzhen Hospital, from 2006 year to 2012 year. The clinical and pathologic features were reviewed. All cases were histochemieal stained in elasticity/van Gieson, AB/PAS and Masson. Three ca- ses of aortic tumors were immunohistochemial staining for SMA,CD31,CD34 monoelonal antibodies. All cases were analyzed histological changes at aortic wall compositions. Results： Aortic wall histological structures were abnormal. They were altered in elastic lamellae, smooth muscle cell layers and extraeellular matrix （ ECM ）. Three cases were formed aortic tumors. The pathologic diagnosis of one case is leiomyoma in aortic wall; the other two were tumor-like lesions. Conclusion ： Aortic wall remodeling was observed in some cases of aortic aneurysms, aortic dissections and Marfan syndrome. The reason for vessel remodeling is alterations of different compositions in the aortic wall. Some aortic neoplasm may be formed by one composition hyperplasia in the aortic wall.