眼眶巨细胞血管纤维瘤

目的 探讨眼眶巨细胞血管纤维瘤的组织病理学特征，侧重于诊断和鉴别诊断。方法 采用ＨＥ染色和免疫组化标记，对２例发生于眼眶的巨细胞血管纤维瘤进行临床病理学分析。结果 ２例均表现为眼眶部位缓慢性生长的无痛性肿块。大体上肿瘤境界较清楚，镜下显示特征性改变：肿瘤的主要成份由无结构性增生的圆形、卵圆形和短梭形细胞组成，增生的瘤细胞无异型性；肿瘤内富含血管及内衬不连续性多核巨细胞的裂隙样或血窦样假血管性腔隙；

Giant cell angiofibroma of the orbit

Objective To study the histopathologic characteristics of giant cell angiofibroma with emphasis on diagnosis and differential diagnosis. Methods The clinical and pathological features of two typical cases of orbital giant cell angiofibroma were evaluated by light microscopy and immunohistochemistry. Results Both cases presented as slowly growing, painless masses that located in the orbit. Grossly, they were well-circumscribed tumors. Microscopically, the tumor was composed of a patternless proliferation of round, ovoid or short spindle cells. Both nuclear atypia and mitotic activity were absent. The tumor was characterized by a prominent network of blood vessels and irregular cleft like or sinusoid psendovascular spaces that were lined with a layer of discontinuous multinucleate giant cells. The stroma displayed a variable degree of collagenization or myxoid degeneration, with some areas recapitulating the patternless pattern of solitary fibrous tumor. Immunohistochemically, both the neoplastic cells and multinucleate giant cells expressed CD34 and vimentin. The patients underwent local excision and remained well at the last follow-up. Conclusions Giant cell angiofibroma is a distinctive benign lesion or potentially a tumor of low-grade malignancy, which occurred predominantly in the orbit in adults. Histologically, this rare entity should be distinguished from giant cell fibroblastoma, extrapleural solitary fibrous tumor, multinucleate cell angiohistiocytoma and angiomatoid fibrous histiocytoma.