鼻腔孤立性纤维性肿瘤3例临床病理分析

Solitary fibrous tumor of nasal cavity: a clinicopathological analysis of three cases

目的探讨鼻腔孤立性纤维性肿瘤(SFT)的临床特点、病理诊断及鉴别诊断、治疗与预后。方法回顾性分析3例鼻腔SFT的临床资料、组织学形态和免疫组化标记,并复习相关文献。结果3例SFT均为男性,均发生于右鼻腔。主要症状为鼻塞、流涕。肿块最大径3cm^5cm。镜下见病变位于黏膜下,边界较清,由交替分布的细胞密集区与稀疏区组成,瘤细胞呈梭形或卵圆形,似纤维母细胞,形态温和,无明显异型性,胞质少,淡嗜红色,核呈卵圆形,核仁不明显,偶见核分裂像(<1个/10HPF),组织结构多样,多杂乱排列,局部呈束状或席纹状,间质见大量红染的胶原纤维束沉积。肿瘤组织内血管丰富,管壁薄,局部呈裂隙状,形成血管外皮瘤样结构,其中1例局部血管壁增厚,玻璃样变性。3例均未见坏死。免疫组化示3例肿瘤细胞CD34、STAT6、CD99、BCL-2均阳性,S100、SMA阴性,术后随访6~54个月,未见复发。结论发生于鼻腔的孤立性纤维性肿瘤(solitary fibrous tumor,SFT)少见,应注意与该部位的其他梭形细胞肿瘤鉴别,该肿瘤属于中间性肿瘤,局部可复发,偶见转移,应对其进行长期密切随访。

Objective To study the clinical features,pathological diagnosis,differential diagnosis,treatment and prognosis of solitary fibrous tumors ( SFT) of the nasal cavity.Methods The clinicopathological and immunohistochemical features were reevaluated in 3 cases of SFT of nasal cavity,with review of the relevant literature.Results All 3 cases of SFT were male and all occurred in the right nasal cavity.The main symptoms were nasal obstruction and discharge.SFT ranged in size from 3 cm to 5 cm.Microscopically,the tumors were submucosal,and border clear.They were composed of alternately distributed cell dense areas and sparse areas.The tumor cells were spindle or oval,like fibroblast,mild form, no obvious atypia,various tissue structures,and arranged in local fascicular or storiform.A large number of red collagen fibrils were deposited in the interstitium.The tumors were rich in blood vessels,focal thin-walled,and hyalinization changes in one case.No necrosis was found in all 3 cases.Immunohistochemical study showed that tumor cells were positive for CD34,STAT6,CD99 and BCL-2,while S100 and SMA were negative.Conclusion The solitary fibrous tumor occurring in the nasal cavity is rare and it should be distinguished from other spindle cell tumors of the site.SFT belongs to the intermediate tumor.It can be locally recurrent,occasionally metastasic,and should be followed up for a long time.