巨细胞纤维母细胞瘤的临床病理学分析

Giant cell fibroblastoma in childhood:a chinicopathologic analysis of 5 cases

目的总结巨细胞纤维母细胞瘤(giant cell fibroblastoma,GCF)的临床病理学特征及生物学行为,探讨其诊断和鉴别诊断方法。方法回顾性分析5例GCF的临床表现及病理学形态,采用免疫组织化学Envision法检测其免疫学表型。结果5例患儿均为男性,临床表现为躯干及下颌部皮下缓慢生长的无痛性结节。肿块平均约2～3.5cm大小,镜下病变主要位于真皮层内并向深部皮下脂肪组织内浸润性生长,主要由成束的梭形细胞组成,特征性形态表现为不规则分布的裂隙样或扩张的脉管样腔隙结构,其内可见散在的深染多核巨细胞;免疫组化标记显示梭形细胞和多核巨细胞CD34(+)、p75(+)和ApoD(+),S-100蛋白(-)、FXIIIa(-)和a-SMA(-)。结论GCF好发于婴幼儿,为中间性软组织肿瘤,宜采取局部广泛切除术,病理学特征和免疫组织化学标记是诊断和鉴别诊断的依据。

Objective To investingate the clincopathologic characteristics of giant cell fibroclastoma (GCF), with emphasis on diagnosis and differential disgnosis. Methods The clinical, pathological and immunohistochemical features of 5 cases were analysed retrospectively. Results All patients are males. The tumors, with mean size of 2 ~ 3.5 cm, presented as slowly growing painless nodules, located in the trunk. Histologically, the tumors were mainly located in the epidermis layer and to the deep subcutaneous tissue i...