目的　研究巨细胞纤维母细胞瘤的临床病理学特点和免疫组织化学表型 ,探讨其鉴别诊断及组织学起源。方法　采用光镜观察结合免疫组织化学LSAB法标记对 7例巨细胞纤维母细胞瘤进行分析。结果　 7例患者中 6例为儿童 ,1例为 35岁成年人。男性 5例 ,女性 2例。临床上主要表现为躯干和四肢皮下缓慢增大的无痛性结节 ,平均直径 2 .9cm。镜下显示 ,肿瘤境界不清 ,主要位于真皮层。瘤细胞主要由轻至中度异型的梭形细胞组成 ,多呈疏松的束状或波浪状排列 ,间质呈纤维黏液样 ,部分区域呈致密胶原化。本病的特征性形态表现肿瘤内含有一些不规则分布的裂隙样或扩张窦样的假脉管性腔隙 ,其腔隙面内衬一层不连续的梭形细胞和核深染多核巨细胞 ,两种细胞在形态上有移行。免疫组织化学标记显示梭形细胞和多核巨细胞均表达波形蛋白和CD34。 5例附有随访资料 ,其中 2例术后复发。结论　 (1)巨细胞纤维母细胞瘤是一种好发于儿童的中间性纤维母细胞性肿瘤 ,较易局部复发 ,掌握其独特的临床病理学特征对避免误诊为一些具有相似形态的病变具有重要意义 ;(2 )巨细胞纤维母细胞瘤与好发于成人的隆突性皮纤维肉瘤在临床表现、免疫组织化学、细胞及分子遗传学上均极为相似 ,在组织学上也可共存 ,提示两者在组织学发生上关系密切 , Objective To study the clinicopathological characteristics and immunohistochemical phenotype of giant cell fibroblastoma and to explore its differential diagnosis and histological origin. Methods Seven cases of giant cell fibroblastoma were analyzed by light microscope and immunohistochemistry LSAB method. Results Of the 7 patients, 6 were children and 1 was 35-year-old adults. 5 males and 2 females. The main clinical manifestations of the trunk and limbs slowly increased subcutaneous nociceptive nodules, the average diameter of 2. 9cm. Microscopically, the tumor is not clear, mainly in the dermis. The tumor cells mainly consisted of mild to moderately shaped spindle cells, mostly loosely arranged in bundles or waves, with interstitial fibroids in the interstitium and dense collagen in some areas. The morphological features of the disease showed the tumor contains some irregular distribution of crack-like or dilated sinusoid pseudo-vascular lacunar, the lacunar surface lined with a layer of non-continuous spindle cells and nuclear deep-stained multicellular giant cells Both cells migrated in morphology. Immunohistochemical staining showed that both spindle cells and multinucleated giant cells expressed vimentin and CD34. 5 cases with follow-up information, including 2 cases of recurrence. Conclusions (1) Giant cell hemangioblastoma is a kind of intermediate fibroblastic tumor in children. It is easy to local recurrence. It has its own unique clinicopathological features to avoid misdiagnosis as some lesion with similar morphology. Significance; (2) Giant cell fibroblastoma and occurred in adults with protuberant lemma fibrosarcoma in clinical manifestations, immunohistochemistry, cell and molecular genetics are very similar in histology can coexist, Suggesting that both are closely related to the occurrence of histology,