巨细胞纤维母细胞瘤的临床病理特征

Clinicopathologic characteristics of giant cell fibroblastoma

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 study the clinical,pathological and immunohistochemical features of giant cell fibroblastoma（ GCF）,and to discuss its differential diagnosis and histogenesis. Methods Three cases of GCF were collected and investigated by light microscopy and immunohistochemistry,with review of the related literature. Results Three cases occurred in children. Two were male and one was female. Clinically,all cases appeared as slow-growing painless nodules located in the dermis or subcutis of the trunk,with a mean diameter of 2. 2 cm. Microscopically,the tumor was poorly circumscribed and was composed of slightly to moderately atypical spindle cells which were arranged loosely in fascicular or wavy manner,embedded in a fibromyxoid background and collagenous in partial area,with occasionally finding of multinucleated giant cells. The morphological features were characterized by irregular distributed cleft-like or sinusoid-like pseudovascular spaces,lined by a discontinuous row of spindle and multinucleate giant cells with hyperchromatic nuclei.Immunohistochemically,both cells expressed vimentin and CD34. Follow-up information in all three cases showed no local recurrences. Conclusions GCF is a distinctive fibroblastic tumor of intermediate malignancy that primarily affects children,and easy to have local recurrences. Recognizing its clinical and pathological characteristics is important to avoid misdiagnosis with other lesions with similar features.