韧带样型纤维瘤病的临床病理分析

目的：探讨韧带样型纤维瘤病（desmoid-type fibromatosis,DTF）的临床病理特征,提高对DTF的认识和诊断水平。方法：分析14例DTF的临床特征,HE及免疫组化观察其病理学特征。结果：14例DTF均为女性,年龄22~81岁,中位数31.5岁,12例发生于腹部,右侧胸壁2例。光镜下以纤细的梭形细胞与胶原纤维交错排列为特征,细胞无异型性,核分裂像罕见;免疫组化Vimentin和β-catenin阳性,部分表达SMA,不表达S-100、CD34、CD117、Dog-1。手术切除随访4~113个月,1例复发。结论：DTF好发于年轻女性,需要与多种良、恶性梭形细胞肿瘤鉴别,细胞核表达β-catenin对DTF诊断和鉴别诊断有重要价值,手术切除是主要治疗手段,切缘残瘤有较高的复发风险。

The analysis of clinicopathologic features in desmoid-type fibromatosis

Objective： To explore the clinicopathologic features of desmoid-type fibromatosis（DTF）, and to improve the recognition and diagnostic ability for DTF. Methods： The clinicopathologic features of 14 cases DTF were analyzed, and the pathological features of the tumour were observed by using HE staining and immunohistochemistry（IHC）. Results： 14 cases DTF were all female, and the median age was 31.5 years old（range 22-81 years old）, 12 cases DTF were found in abdominal, and 2 in the right chest wall. In light microscope, the histologic features of DTF showed the proliferation of bundles of slide spindle cells across the collagenous fibres, cytological atypia was absent and pathological karyokinesis was rare. IHC showed a diffuse positive for Vimentin and β-catenin, partly for SMA, and negated for S-100, CD34, CD117 and Dog-1. Patients were followed up from 4 to 113 months after DTF resection, 1 case recurred. Conclusion： DTF is usually seen in young female, and needs to differentiate from many benign and malignant spindle cell tumors. It is of most importance for diagnosis and differential diagnosis of DTF that β-catenin is positive in the nuclear and cytoplasm. The main therapy is resection of DTF by operation, and the positive margins after operation may be indicate high recurrence risk.