臀部巨大韧带样纤维瘤一例及文献复习  

Desmoid tumor:one case report of hip huge desmoid tumor and literature review

作　　者：唐成林[1] 柳万国[1] 孙莉[1] 徐峰[1] 佘鹏[1] 李云峰[1] 边帅[1] 

机构地区：[1]吉林大学第二白求恩临床医学院骨科,吉林长春130041

出　　处：《罕少疾病杂志》2011年第1期44-47,共4页Journal of Rare and Uncommon Diseases

摘　　要：目的探讨韧带样纤维瘤发病机理、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,并进行了有关文献复习。结果报道臀部巨大韧带样纤维瘤一例并复习相关文献。本病例患者,女,45岁,临床表现为左臀部胀痛、肿块和跛行,无创伤史和家族性遗传病史。病变位于左侧股骨后方自髋臼顶水平至股骨髁水平,呈条索状大小约28cm×4cm×3cm。B超检查表现为边界清楚中高回声软组织影。CT检查示边界较清的均匀稍低密度的异常软组织影。MRI检查中T1WI中央部分为极低信号影,其周边为低信号影,病灶边界清晰;T2WI呈边界清晰混杂等低信号,而在T2WI脂肪抑制像病灶呈混杂高信号,病灶边界欠清。患者行左臀部占位性病变边缘性切除术,术中见肿物无包膜边界不清且呈蟹足样侵犯周围组织。术后病理诊断为韧带样纤维瘤,随访3月无不适主诉和复发。结论韧带样纤维瘤是一种病因不明的来源于间叶组织的具有局部侵袭特征且极易复发的软组织肿瘤,临床表现不典型,影像学表现具有一定特异性,确诊依靠病理检查诊断,目前仍以手术治疗为主,放疗和药物治疗为辅。Objective To study the etiopathogenesis,clinical and radiographic features and treatment of desmoid tumor.Methods A case report of desmoid tumor and literature review.Results A 45 year old woman had huge desmoids tumour with slight pain,lump and lame in the left hip site and without trauma and inherited disease.The size of this tumor was about 28cm in length,4cm in width and 3cm in thickness from acetabular level to condyle of femur.Ultrasound examination showed moderate or high-level echo of this lesion with negative margin.The lump manifested homogeneous low density with well defined margin in the computed tomography scan.The lump manifested extremely hypointense in central region surrounding by hypointense rim with negative margin in T1WI and low-moderate mixed signal in T2WI,but high mixed signal with ill-defined margin in STIR image.Pathological examination showed a typical desmoid tumour.Wide local excision was performed and found lump haven＇t not capsule and local infiltration in operation.The outcome was effective without complaint and recurrence after fellowed up for three months.Conclusion Desmoid tumor is a soft tissue neoplasm that arises from fascial and musculoaponeurotic tissues and local infiltration with high recurrence rate.The etiopathogenesis of desmoid tumor is still unknown.It has atypical clinical presentations and some radiographic features.Its diagnosis depends on pathological examination.The main treatment of desmoid tumor is surgery and followed by radiotherapy and pharmacological treatment.

关 键 词：韧带样纤维瘤 病因 影像学 诊断 治疗 

分 类 号：R730.262[医药卫生—肿瘤]