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目的观察巨细胞纤维母细胞瘤临床病理学及免疫组化表型的特征,探讨其鉴别诊断和可能的组织学起源。方法收集3例巨细胞纤维母细胞瘤,采用苏木精-伊红染色进行光镜观察,采用EnVision两步法进行免疫组化分析,并复习相关文献。结果 3例患者均为儿童,男性2例,女性1例。临床上主要表现为躯干真皮及皮下缓慢增大的无痛性结节,平均直径2.2 cm。镜下显示肿瘤境界不清,主要位于真皮及皮下。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,部分区域胶原化,偶见散在、多核巨细胞。特征性形态表现为肿瘤内含有一些不规则分布的裂隙样或扩张窦样的血管样腔隙,其腔隙面内衬一层不连续的梭形细胞和核深染的多核巨细胞。免疫组化示梭形细胞、多核巨细胞vimentin和CD34(+)。3例均附有随访资料,术后无复发。结论巨细胞纤维母细胞瘤是一种好发于儿童的与隆突性皮肤纤维肉瘤密切相关的中间性(局部具有侵袭性)或低度恶性的纤维母细胞性肿瘤,局部易复发,掌握其独特的临床病理学特征,对避免误诊为相似形态的病变具有重要意义。
OBJECTIVE: To observe the clinicopathological and immunohistochemical features of human cytofibroblastic tumor and explore its differential diagnosis and possible histological origin. Methods Three cases of giant cell fibroblastoma were collected and observed by light microscopy with hematoxylin-eosin staining. EnVision two-step immunohistochemical analysis and review of relevant literature were performed. Results All three patients were children, including 2 males and 1 females. The main clinical manifestations of the trunk of the dermis and subcutaneous slowly increased painless nodules, with an average diameter of 2.2 cm. Microscopic tumor showed unclear, mainly located in the dermis and subcutaneous. Tumor cells are mainly composed of mild to moderately shaped spindle cells, mostly loose bundles or wavy arrangement, interstitial fibrous myxoid, some areas of collagen, occasionally scattered, multinucleated giant cells. The characteristic appearance of the tumor is that there are some vascular-like cavities with irregularly distributed fissure-like or dilated sinusoids in the tumor, and the lacunar surface is lined with a discontinuous spindle cell and a nuclear-stained multicellular giant cell. Immunohistochemistry showed spindle-shaped cells, multinucleated giant cells vimentin and CD34 (+). 3 cases were accompanied by follow-up information, no recurrence after surgery. Conclusions Giant cell fibroblastoma is an intermediate (locally invasive) or low-grade fibroblastic tumor that is closely related to procidant dermatofibrosarcoma in children. It is locally easy to relapse and masters Unique clinical and pathological features, to avoid misdiagnosis of similar morphological lesions of great significance.