掌纤维瘤病9例临床病理特征

Clinicopathologic characteristics of 9 cases of palmar fibromatosis

共纳入9例掌纤维瘤患者,其中男5例,女4例,发病年龄17~68岁,发病部位以掌尺侧多见。肿瘤大体均为单结节状,大小0.8~2.2(平均1.3)cm,切面实性灰白灰黄色。镜下病变由增生的梭形纤维母细胞和不等量的胶原纤维组成,呈束状排列,浸润筋膜组织,核分裂罕见。免疫组织化学显示梭形细胞均表达Vimentin及SMA,Calponin,MSA等肌源性标志物,β-catenin有5例核阳性、4例阴性(核阳性率56%),而Desmin,S-100,CD34,EMA均阴性,Ki-67增殖指数小于2%。7例获得随访2~40个月,1例复发。掌纤维瘤病是一种较少见的特殊类型的纤维瘤病,诊断及鉴别诊断需靠临床症状、组织学形态及免疫表型特点,合适的外科手术治疗具有较低的复发率。

The clinical pathological data of 9 cases of palmar fibromatosis were collected.The 9 cases included 5 males and 4 females with ages range from 17 to 68 years.All cases consist of single nodule with an average diameter of 1.3 cm(range from 0.8 to 2.2 cm)which usually predominate on the ulnar side of the palm.On cut section revealed firm and gray-yellow to gray-white surface.Microscopically,the nodules were composed of a monotonous,variably collagenous,fascicular proliferation of bland,uniform fibroblasts which infiltrated fascial tissue,mitosis was rare.The tumor cells expressed Vimentin and muscle-derived markers such as SMA,Calponin,and MSA.Aberrant nuclear positive staining forβ-catenin was observed in 5 cases and the others were negative(nuclear positive rate 56%).The cells were negative for Desmin,S-100,CD34,and EMA.The proliferation index of Ki-67 was less than 2%.Only one case developed local recurrence during 2–40 months of follow-up.Palmar fibromatosis is a rare and special type of fibromatosis.The diagnosis and differential diagnosis depend on clinical symptoms,histological morphology,and immunophenotypes.Suitable surgical treatment has a low recurrence rate.