摘要
目的探讨原发性动脉内膜肉瘤(intimal sarcoma,IS)的临床表现、影像学、组织病理学特征、诊断及治疗。方法收集17例国内IS(本院报道1例,文献报道16例)的临床资料,对其主要临床表现、影像学检查、病理学诊断及鉴别诊断、治疗与预后等特征进行分析。结果内膜肉瘤主要发生于成年人,女性略多见,好发于肺动脉。大部分以呼吸系统非特异性表现为主要症状,可伴有胸闷,痰中带血。体检常出现肺呼吸音异常等。心脏超声检查主要表现为病变区回声团块;CT显示为充盈缺损改变。术中所见有助于判断肿瘤性质,但最后确诊依赖于术后组织病理学检查和免疫组织化学染色。光镜下肿瘤组织主要由梭形细胞构成,并见不同比例的多核瘤细胞、上皮样或星芒状瘤细胞,间质常伴有不同程度的黏液样变;免疫组化检测瘤细胞vimentin弥漫强阳性,SMA、CD34等部分表达。17例其中12例随访3～27个月,5例死亡。结论内膜肉瘤是一种罕见的具有纤维母细胞或肌纤维母细胞分化的恶性间叶性肿瘤,发生于体循环和肺循环大动脉壁,分化低、预后差。明确其发病部位、影像学特征及临床病理有助于正确诊断。
Objective To explore the clinical, treatment progress of artery intimal sarcoma tic intimal sarcoma were collected in which 1 other 16 cases were reviewed from literature maging, histopathological features,diagnosis and （IS）. Methods Clinical data of 17 cases of domes- case was diagnosed in Hubei Cancer Hospital and Main clinical manifestations, image examination, , pathological diagnosis and differential diagnosis, treatment and prognosis features were ana- lyzed and summarized. Results Intimal sarcoma occurred mainly in adults, slightly more com- mon in female, predominantly from the pulmonary artery. Most of cases showed the non-spe- cific manifestations of respiratory system as the main symptoms, along with problems such as chest tightness, bloody sputum. The physical examinations often detected abnormal lung breath sounds. Echocardiography mainly showed mass with echoes in the lesion. CT showed the filling defect changes. The intraoperative findings helped determine the nature of the tumor, but the final diagnosis depends on the postoperative histopathological examination and immunohistochemical staining. Under microscope, tumor tissue was mainly composed of spin- dle cells with different proportions of multi-nuclear, epithelioid or stellate-shaped tumor cells.The interstitial was often accompanied by varying degrees of myxoid change. Immunohisto- chemical detection of tumor cells were diffuse strong positive of vimentin, partial expression of SMA and CD34. 12 patients were followed up for 3 to 27 months and five of them died. Cen- clusion Intimal sarcoma is a rare malignant mesenchymal tumor with the differentiation of fibro- blasts or myofibroblasts, occurring in the aorta wall of the systemic and pulmonary circulation. The differentiation is low and prognosis is poor. Definition of the disease lesion, imageology characteristics and clinical pathology contribute to the correct diagnosis.
出处
《湖北民族学院学报（医学版）》
2012年第3期16-21,共6页
Journal of Hubei Minzu University(Medical Edition)
关键词
内膜肉瘤
动脉
临床病理
影像学
治疗
intimal sarcoma
artery
clinical pathology
imageology
treatment
