胸部韧带样型纤维瘤一例及文献分析  

One Case Report and Literature Analysis of Chest Desmoid-Type Fibromatosis

作　　者：周绮华[1] 廖鹏飞[1] 冯浩坚 欧陕兴[2] 刘盼丽 

机构地区：[1]广州市番禺区中医院CT室,广东广州511400 [2]广州军区广州总医院放射科,广东广州510010

出　　处：《黑龙江医学》2017年第9期878-880,共3页Heilongjiang Medical Journal

摘　　要：目的探讨韧带样型纤维瘤发病机理、临床特点、影像学特征及治疗方法。方法报告1例这种罕见病例,总结相关病例报告,并进行了相关文献分析。结果报道胸壁巨大韧带样型纤维瘤一例并分析相关文献。本病例患者,女,19岁,体检发现左胸包块,临床无明显症状,无创伤史和家族性遗传病史。胸片示左胸包块;CT示:左侧胸腔密度不均匀的不规则肿块,邻近肋骨呈虫蚀样骨质破坏。患者行左胸部占位性病变并部分肋骨切除术,术中见肿物包膜完整,侵犯左侧第6~9肋骨。术后病理诊断为韧带样型纤维瘤,随访1个月无不适主诉和复发。结论韧带样型纤维瘤是一种病因不明的来源于间叶组织的肿瘤,具有局部侵袭特征,且极易复发,临床表现不典型,影像表现具有一定特异性,确诊依靠病理诊断,目前仍以手术治疗为主,放疗和药物治疗为辅。Objective To investigate the pathogenesis, clinical features, imaging features and treatment of the desmoid-type fi- bromatosis. Methods Reporting 1 case of this rare case, summarizing the relevant case reports, and analyzing related literature. Results A case of desmoid-type fibromatosis was reported and the related literature was analyzed. This case, female, 19 years old, physical examination found left chest mass, with no obvious symptoms, no trauma history and family history. Chest X-ray showed left chest mass, and CT showed irregular density mass of left pleura1 and adjacent to the rib, which was found destroyed. Patients under- went left breast lesions and partial rib resection, intraoperative saw the tumor has integrity capsule, the left side of the 6 -9th rib was invaded. The postoperative pathological diagnosis was desmoid-type fibromatosis, and there was no discomfort and recurrence in one month follow-up. Conclusion Desmoid-type fibromatosis is a disease of unknown etiology which derived from mesenchymal tissue, having locally aggressive features and easy to relapse, having atypical clinical manifestations and specificity imaging characteristics. The final diagnosis depends on pathological diagnosis. Surgery is the main treatment, with radiotherapy and drug therapy as a supple- ment.

关 键 词：胸部 韧带样型纤维瘤 诊断 治疗 

分 类 号：R655.1[医药卫生—外科学]