Yao J M, Zeng H Y, Tan Y S, Hou Y Y
Department of Pathology, Zhongshan Hospital of Fudan University, Shanghai 200032, China.
Zhonghua Bing Li Xue Za Zhi. 2017 Apr 8;46(4):223-227. doi: 10.3760/cma.j.issn.0529-5807.2017.04.002.
To investigate the clinical features, immunohistochemical and differential diagnosis of desmoplastic fibroblastoma. The clinical data and pathology features of 7 cases of desmoplastic fibroblastoma were collected and immunohistochemical study were carried out in all cases with a review of the literatures. There were 2 males and 5 females, with age ranging from 31 to 71 years (average and mean age were 59 and 61 years, respectively). The tumors were located in extremities and abdomen (left toe and right toe, right foot back, left leg and right thigh, right forearm and left hepatic lobe). Clinically, the tumors presented as slow growing painless masses of long standing duration. Grossly, the tumors were well-circumscribed with firm, white to gray cut-off surface. Tumor size ranged from 1.2 to 4.0 cm in maximum diameter (average 3.0 cm). Microscopically, 2 cases were located in dermis, 4 cases were located in subcutaneous and 1 case was located in liver parenchyma. It was composed of spindle-shaped or stellate cells with a fibroblastic or myofibroblastic appearance, and sparsely scattered in densely fibrous or fibromyxoid background. There was small vascular component in tumor background. At high magnification, the tumor cells were medium size with abundant cytoplasm, and the nucleus were small and always with small nucleoli. In some cases, the tumor cells were slightly larger with enlarged nuclei, but without cellular atypical and mitosis. Immunohistochemical study showed that the tumor cells were strongly positive for vimentin, desmin, S-100 protein and CD34, but CKpan was negative. α-SMA showed focal positive in one case. Ki-67 index ranged from 1% to 2%. Four cases were followed-up (ranged from 11 to 21 months, average 16.5 months) and the patients had no recurrence after surgery. Desmoplastic firoblastoma is a rare soft benign tumor. The differential diagnosis includes other benign or low-grade fibroblastic/myofibroblastic lesions.
探讨促纤维组织增生性纤维母细胞瘤的临床特征、免疫组化表现及鉴别诊断。收集7例促纤维组织增生性纤维母细胞瘤的临床资料及病理特征,对所有病例进行免疫组化研究并复习相关文献。其中男性2例,女性5例,年龄31~71岁(平均年龄分别为59岁和61岁)。肿瘤位于四肢及腹部(左足趾、右足趾、右足背、左腿、右大腿、右前臂及左肝叶)。临床上,肿瘤表现为生长缓慢的无痛性肿块,病程较长。大体检查,肿瘤边界清楚,质地硬,切面呈白色至灰色。肿瘤最大直径1.2~4.0 cm(平均3.0 cm)。镜下,2例位于真皮层,4例位于皮下,1例位于肝实质。肿瘤由梭形或星状细胞组成,呈纤维母细胞或肌纤维母细胞样外观,稀疏分布于致密的纤维或纤维黏液样背景中。肿瘤背景中有少量血管成分。高倍镜下,肿瘤细胞中等大小,胞质丰富,核小,常有小核仁。部分病例中,肿瘤细胞稍大,核增大,但无细胞异型性及核分裂象。免疫组化研究显示,肿瘤细胞波形蛋白、结蛋白、S-100蛋白及CD34均呈强阳性,细胞角蛋白(CKpan)阴性。α-平滑肌肌动蛋白(α-SMA)在1例中呈局灶阳性。Ki-67指数为1%~2%。4例进行了随访(随访时间11~21个月,平均16.5个月),患者术后无复发。促纤维组织增生性纤维母细胞瘤是一种罕见的良性软组织肿瘤。鉴别诊断包括其他良性或低级别纤维母细胞/肌纤维母细胞性病变。