巨细胞纤维母细胞瘤误诊1例并文献复习

Giant Cell Fibroblastoma:Report of One Misdiagnosed Case and the Literature Review

目的 :观察巨细胞纤维母细胞瘤病理形态及免疫组化特点 ,并探讨其鉴别诊断及组织来源。方法 :对 1例巨细胞纤维母细胞瘤进行光镜及免疫组化染色。结果 :肿瘤细胞主要由具有轻至中度异型性的梭形细胞组成 ,呈束状或波浪状排列 ,间质粘液样变及胶原化。本病主要的形态特征性为 :出现独特的不规则分布的裂隙或假血管腔 ,内衬多核巨细胞 ,免疫组化 Vimentin、CD34呈阳性表达。结论 :巨细胞纤维母细胞瘤是一种好发于儿童的中间性的软组织肿瘤 ,具有局部复发倾向 ,与成人的隆突性皮肤纤维肉瘤可能同属

Objective:To study the morphological and immunohistochemical features of giant cell fibroblastoma (GCF), with emphasis on its differential diagnosis and histogenesis. Method:One case of GCF was observerd by light microscope and immunohistochemical staining.Results:The tumor was composed of slightly to moderately heterogeneous fusiform cells which were arranged in parallel or wavy manner with myxiod degeneration and collagenation in stroma.The pathognomonic features consisted of irregular distributed cleft or sinusoid-like pseudovascular spaces lined with multinucleate giant cells, and immunohistochemical staining showed Vimentin?CD34 positive.Conclusion:GCF is a distinctive soft tumor of intermediate malignancy that occurs predominantly in children and is susceptible to local relapse. GCF and its adult counterpart dermatofibrosarcoma protuberans (DFSP) possibly belong to the CD34 positive dendritic neoplasms.