眼眶孤立性纤维瘤临床病理分析及鉴别诊断

目的 探讨眼眶孤立性纤维瘤的临床病理学特征、诊断、鉴别诊断及其治疗方法.方法 回顾性系列病例研究.回顾性分析2000年1月至2007年12月在复旦大学附属眼耳鼻喉科医院眼科进行手术并经组织病理学检查确诊的6例眼眶孤立性纤维瘤患者的资料,归纳其临床和影像学特点以及病理和免疫组织织化学学染色结果.结果 男性4例,女性2例,年龄19～57岁.临床表现:3例主诉为眼球突出,3例为球结膜下或眶缘肿块.影像学检查:边界清晰的实质性占位影,均可被造影剂均质增强;肿瘤位于肌锥内间隙者2例,肌锥外间隙者4例.病理学检查:肿瘤细胞呈弥漫分布的梭形,形态规则,核分裂象偶见,胞质透明或轻度嗜酸性,多以血管外皮瘤样不规则单相排列,也可呈席纹状、编织状及束状等,血管丰富,壁薄,可呈鹿角样,间质有玻璃样变的胶原纤维沉积;免疫组织化学染色结果:肿瘤细胞波形蛋白(+),CD34(+),CD99(+).治疗:4例行肿瘤摘除,2例因与重要结构粘连紧密行局部切除.随访:4例无复发,2例复发.结论 眼眶孤立性纤维瘤是一种发生于眼眶的少见肿瘤,临床表现、影像学检查和病理学表现极易与其他肿瘤混淆,结合肿瘤的病理学形态及免疫组织化学检查可以确诊,并可作为与其他肿瘤相鉴别的特征之一.(中华眼科杂志,2008,44:691-695)

Orbital solitary fibrous tumor: a clinicopathologic analysis

Objective To study clinicopathologic features, histologic characteristics, differential diagnosis and the treatment of orbital solitary fibrous tumor (SFT). Methods Clinical, radiographic and pathologic findings of 6 cases of SFT were retrospectively analyzed. Immunohistocbemistry were performed on selected samples. Results Four patients were males and 2 were females. Patients age ranged from 19 to 57 years old. The location of the tumor was in the muscle cone(case 1 and case 5), medial(case 3), lateral (case 4) , superior (case 2) and inferolateral (case 6) portion of the orbit, respectively. The presenting symptom was proptosis in 3 cases and was mass of subconjunetival or orbit margin in other 3 cases. Image examination: SFT appeared as a round (case 6 showed irregular) and well-circumscribed parenchymatous mass that could be homogenously enhanced by contrast. Histologically, SFT displayed as a mass of spindle cells in an irregular arrangement. Sometime, tumor cells could be storiform or sarciniform. Mitotic figures were infrequent and usually there were 0 to 3 mitotic figures per 10 high-power fields. Hyalinization and staghornform blood vessels were frequently observed. SFT was immunoreactive for markers such as Vim,CD34 and CD99. Two cases were recurred. Conclusions SFT is a rare orbital tumor and could be confused with other types of orbital tumors. This tumor can be diagnosed by pathological and immunocytochemical studies, these characteristics can be used to differentiate it from other types of orbital tumors. (Chin J Ophthalmol,2008, 44:691-695)