伴有巨细胞的血管外皮细胞瘤／孤立性纤维性肿瘤的临床病理观察

Clinicopathologic characteristics of hemangiopericytoma/solitary fibrous tumor with giant cells

目的探讨伴有巨细胞的血管外皮细胞瘤／孤立性纤维性肿瘤的病理学特点、诊断及鉴别诊断要点。方法采用HE及免疫组织化学（EnVision法）方法，对7例分别发生于眼眶及眶外的伴有巨细胞的血管外皮细胞瘤／孤立性纤维性肿瘤进行病理学分析。结果2例为发生于眼眶的肿瘤，其中l例为复发病例；5例为眶外肿瘤。镜下特征：肿瘤境界清楚，由无明显异型性的圆形或梭形的肿瘤细胞与胶原混杂组成，富含血管，部分区域形成假血管腔隙样结构，多核巨细胞衬于腔隙的内壁或散布于间质中。免疫组织化学标记显示肿瘤细胞和巨细胞表达CD34。7例均行肿块切除术，术后随访4例，均无复发。结论伴有巨细胞的血管外皮细胞瘤／孤立性纤维性肿瘤是一种眼眶和眶外组织的中间型软组织肿瘤，局部切除多可治愈，组织学上需与巨细胞纤维母细胞瘤、软组织多形性透明变性血管扩张性肿瘤、血管瘤样型纤维组织细胞瘤相鉴别。

Objective To study the pathological characteristics, diagnosis and differential diagnoses of hemangiopericytoma-solitary fibrous tumor with giant cells. Methods Pathological characteristics of seven cases of orbital and extraorbital hemangiopericytoma-solitary fibrous tumors with giant cells were evaluated by HE and immunohistochemistry （EnVision method）. Results Two cases were located in the orbit, one of which had recurred. Five cases were located in the extraorbital regions. Histologically, the tumors were well-circumscribed and composed of non-atypical, round to spindle cells with collagen deposition in the stroma. The tumors had prominent vasculatures and in areas, pseudovascular spaces lined by muhinucleated giant cells lining which were also present in the stroma. Immunohistochemically, both neoplastic cells and multinucleate giant cells expressed CD34. Seven patients underwent tumor excision and were well and without tumor recurrence upon the clinical follow-up. Conclusions Hemangiopericytoma- solitary fibrous tumor with giant cells is an intermediate soft tissue tumor. It typically involves the orbital or extraorbital regions. Histologically, the tumor should be distinguished from giant cell fibroblastoma, pleomorphic hyalinzing angiectatic tumor of soft part and angiomatoid fibrous histiocytoma.