中国组织化学与细胞化学杂志CHINESE JOURNAL OF HISTOCHEMISTRY AND CYTOCHEMISTRY第29卷第6期2020年12月V ol.29.No.6December.2020〔收稿日期〕2020-06-13 〔修回日期〕2020-12-10〔作者简介〕李欢欢，女（1982年），汉族，主管技师*通讯作者(To whom correspondence should be addressed)：****************巨细胞纤维母细胞瘤一例李欢欢，王倩，李巧转*（解放军联勤保障部队904医院病理科，无锡，214044）〔摘要〕目的 探讨巨细胞纤维母细胞瘤（giant cell fibroblastoma ，GCF ）的临床病理特征、诊断及鉴别诊断。
方法 HE 染色及免疫组织化学染色分析1例GCF 的临床病理特点和免疫表型，荧光原位杂交（FISH ）检测PDGFB 基因易位，并复习相关文献。
结果 患者女童，1岁3个月，因“发现右膝关节外侧包块5个月”入院。
病理学检测示：肿瘤边界不清，浸润周边脂肪组织，瘤细胞梭形，束状或波浪状排列，疏密不均，轻—中度异型，间质呈纤维黏液样，部分明显胶原化，可见核深染的多核巨细胞，核重叠样或位于胞质周边呈花环样。
可见特征性不规则分布的扩张脉管样或裂隙样腔隙，内衬核深染多核巨细胞或多形性梭形细胞。
免疫组织化学染色显示肿瘤细胞呈CD34、CD99阳性，而AE1/AE3、SMA 、desmin 、S-100、CD56、BCL-2、CD68阴性，肿瘤细胞增殖指数低（Ki-67 阳性率3%）。
FISH 检测显示有PDGFB 基因相关易位。
会诊结果诊断为（右膝外上方）巨细胞纤维母细胞瘤。
结论 巨细胞纤维母细胞瘤是罕见的软组织肿瘤，病理容易误诊为其他病变，应加深对其临床病理特征的认识。
〔关键词〕巨细胞纤维母细胞瘤；病理学特征；鉴别诊断〔中图分类号〕R365 〔文献标识码〕A DOI ：10.16705/ j. cnki. 1004-1850. 2020. 06. 011A case report of giant cell fibroblastomaLi Huanhuan, Wang Qian, Li Qiaozhuan *(Department of Pathology, The 904 Hospital of PLA, Wuxi, Jiangsu 214044, China)〔Abstract 〕Objective To investigate the pathological characteristics, diagnosis and the differential diagnosis of giant cell fi -broblastoma (GCF). Methods The clinical pathological characteristics and immunophenotype of one case of GCF were analyzed by HE and immunohistochemistry staining, PDGFB gene translocation was detected by fluorescence in situ hybridization （FISH ）, and related literature was reviewed. Results The fifteen-month-old girl was hospitalized five months after finding a mass in her right knee. Under microscope, pathological examination of the specimen showed the tumor had a obscure boundary and infiltrates into surrounding fatty tissue. The oncocytes were arranged in fascicular or wavy shape with an uneven density, and showed mild to moderate atypia. The interstitium was fibrous mucinous and some of it was obviously collagenized. Multinucleated giant cell could be observed with hyperchromatic nuclei overlapping or garland - like around the cytoplasm. Furthermore, there was a characteristic irregular distribution of dilated vasculature or fissure like cavities lined by multinucleated giant cells with hyperchromatic nuclei or pleomorphic spindle cells. Immunohistochemical staining showed that tumor cells were positive for CD34 and CD99, while negative for AE1 / AE3, SMA, desmin, S-100, CD56, Bcl-2 and CD68, and the proliferation index of tumor cells was low (the positive rate of Ki-67 was 3%). FISH assay detected PDGFB gene translocation. The consultation finally diagnosed this mass as the giant cell fibroblastoma . Conclusion GCF is relatively rare in soft tissue tumors, and easily misdiagnosed as other diseases. Its clinical pathological characteristics should be better cognized .〔Keywords 〕Giant cell fibroblastoma; pathologic feature; differential diagnosis巨细胞纤维母细胞瘤（giant cell fibroblastoma ，GCF ）是罕见的软组织肿瘤，文献报道多发于婴幼儿，由于其比较少见，病理学上容易误诊为其他病变。
