Purpose To explore the clinicopathologic fea-tures, diagnosis and differential diagnosis of desmoid-type fibro-matosis (DTF). Methods The clinicopathological data of 55 cases of DTF were retrospectively analyzed and followed up. Re-sults 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 mitot-ic figures were rare. Immunohistochemical results showed posi-tive 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 ex-pressed. Ki-67 proliferation index was low. The effect of surgical resection was good for DTF. After 2-82 months follow-up, 17 ca-ses recurred and the remainders were disease-free survival. Con-clusion DTF is a rare intermediate tumor of soft tissue with no specificity of clinical and imaging features, which should be dif-ferentiated with a variety of benign and malignant diseases. The nuclear expression of β-catenin acts an important role in diagno-sis and differential diagnosis of DTF.%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.
展开▼
