韧带样型纤维瘤病55例临床病理分析

Analysis of clinicopathologic features in 55 cases of desmoid-type fibromatosis

目的探讨韧带样型纤维瘤病(desmoid-type fibromatosis,DTF)的临床病理特征、诊断及鉴别诊断。方法回顾性分析55例经手术切除诊断为DTF的临床病理资料并复习相关文献。结果 55例中男性18例,女性37例。年龄6~71岁,中位年龄33岁。发生于腹壁者19例、腹壁外者23例、腹腔内和肠系膜者13例。镜检:肿瘤由增生的梭形纤维母细胞与数量不等的胶原纤维构成。细胞无明显异型性,核分裂象罕见。免疫表型:β-catenin核表达,vimentin阳性,部分表达SMA、desmin,不表达CD34、CD117、DOG1及S-100,Ki-67增殖指数较低。手术切除效果好。术后随访2~82个月,17例复发,其余均无瘤生存。结论 DTF是一种少见的软组织中间型肿瘤,临床与影像学特征无特异性,确诊需结合组织病理学及免疫组化标记,β-catenin核表达对DTF的诊断和鉴别诊断有重要价值。

Purpose To explore the clinicopathologic features,diagnosis and differential diagnosis of desmoid-type fibromatosis（DTF）.Methods The clinicopathological data of 55 cases of DTF were retrospectively analyzed and followed up.Results Among 55 cases of DTF,18 cases were male and 37 cases were female.Median age was 33 years old（range from 6-71）.19 cases were occurred in abdominal wall,23 cases were extra-abdomen,13 cases in intra-abdomen and mesentery.DTF was composed of proliferating spindle fibroblasts and a number of collagen fibers.The cell showed no obvious atypia and the mitotic figures were rare.Immunohistochemical results showed positive expression of vimentin,partial expression of SMA,desmin.The positive expression of β-catenin was located in nucleus of DTF cells.CD34,CD117,DOG1,S-100 were negatively expressed.Ki-67 proliferation index was low.The effect of surgicalresection was good for DTF.After 2-82 months follow-up,17 cases recurred and the remainders were disease-free survival.Conclusion DTF is a rare intermediate tumor of soft tissue with no specificity of clinical and imaging features,which should be differentiated with a variety of benign and malignant diseases.The nuclear expression of β-catenin acts an important role in diagnosis and differential diagnosis of DTF.