先天性纤维肉瘤的临床病理分析及文献复习  

Congenital fibrosarcoma:a clinicalpathological study with literature review

作　　者：黄海花[1] 吴秀浅[2] 郑志超[1] 张薇[1] 

机构地区：[1]汕头大学医学院第二附属医院病理科,广东汕头515041 [2]汕头大学医学院附属肿瘤医院介入科,广东汕头515041

出　　处：《现代肿瘤医学》2013年第8期1858-1862,共5页Journal of Modern Oncology

基　　金：广东省自然基金项目(编号:10151503102000029);汕头市重点科技项目资助(编号:汕府科[2009]70号-2)

摘　　要：目的:探讨先天性纤维肉瘤的临床病理改变、分子遗传学特点、诊断、鉴别诊断、治疗及预后。方法:分析3例先天性纤维肉瘤的临床资料、组织形态学特点及免疫组织化学表型,结合文献复习。结果:三个病例的原发部位分别为耳廓、左前臂及腹膜后,发病年龄均在5岁以内。术前均未能明确诊断,其中一例误诊为血管瘤。治疗以手术切除为主,其中一例术前化疗。随访至今,除一例局部复发行截肢术外,其余两例未发现复发或转移。肿瘤细胞均表达Vimentin,部分表达SMA或AAT。结论:先天性纤维肉瘤是发生于婴幼儿的少见的软组织肿瘤,具有独特的分子遗传学改变,治疗以局部扩大切除加化疗为主,虽易复发,但预后较好。Objective：To study the clinicopathological alterations,molecular genetics features,treatment,differential diagnosis and prognosis of congenital fibrosarcoma.Methods：To analyze the clinical materials,morphological features,immunochemistry phenotype of 3 cases of congenital fibrosarcoma,and review the literature.Results：The primary site 3 cases was auricle,left forearm and retroperitoneal respectively.The age of these patients was less than 5 years old.Preoperative diagnosis was not accurate,one initially misdiagnosed as hemangioma.Treatment was mainly surgery,one case received chemotherapy before operation.These three cases were followed up,except one case received partial complex amputation,the others did not found recurrence or metastasis.The expression of Vimentin was found in all tumor cells,the expression of SMA or AAT was found in some cells.Conclusion：As a rare soft tissue malignancy occurs in axial and extremity locations,congenital infantile fibrosarcoma has the sole features of molecular genetics alteration.Therefore,completely local resection or local expand resection with chemotherapy is always given priority to the treatment,and the prognosis is good though it easy relapse.

关 键 词：先天性纤维肉瘤 临床病理 诊断 治疗 预后 

分 类 号：R730.262[医药卫生—肿瘤]