[Clinical and pathological analysis of 35 cases of ocular adnexal solitary fibrous tumor].

Objective: To analyze the clinical and pathological characteristics and prognosis of 35 cases of solitary fibrous tumor of ocular adnexal (SFT). Methods: This was a retrospective case series study. The clinical data of 35 cases of ocular adnexal SFT was collected in Tianjin Eye Hospital from January 2000 to December 2020. The clinical manifestations, imaging examination results, pathological characteristics, treatment of patients were analyzed, and patients were followed up. All cases were classified according to the 2013 classification of World Health Organization of tumors of soft tissue and bone. Results: There were 21 males (60.0%) and 14 females (40.0%). The age range was 17 to 83 years, and the median age was 44 (35, 54)years. All patients were unilateral, with 23 (65.7%) in the right eye and 12 (34.3%) in the left eye. The course of disease ranged from 2 months to 11 years, with an a median duration of 12(6,36)months. Clinical manifestations included exophthalmos, limited eye movements, diplopia, and tearing. All patients underwent surgical treatment of complete resection of the tumor. Ocular adnexal SFT mostly occurred in the upper orbit (19 cases, 73.1%). On imaging examination, the tumor showed well-circumscribed space occupying lesion that heterogeneously enhanced with contrast, and abundant blood flow signals in the tumors. MRI showed isointensity or low signal on T1WI, and significantly enhanced on T2WI, presenting intermediate-to-high heterogeneous signals. The tumor diameter was 2.1 (1.5, 2.6) cm. There were 23 cases (65.7%) of classic subtype, 2 cases (5.7%) of giant cell subtype, 8 cases (22.9%) of myxoid subtype, and 2 cases (5.7%) of malignancy. Immunohistochemical staining showed that Vimentin, CD34 and STAT6 were positively expressed in all patients. Twenty-one cases (60.0%) showed positive expression of BCL-2, and Ki-67 positive index ranged from 1.0% to 10.0%. Tumors in this group were all low-risk according to the Demicco risk stratification. Follow-up was available for 25 patients with a duration of 2 years to 14 years and 7 months, and the median follow-up time was 88 (61, 124) months. Two patients relapsed, and no distant metastasis or death was observed. Conclusions: Ocular adnexal SFT mainly presents as a painless, slow-growing mass. And most of them are typical SFT. The imaging manifestations are varied Ocular adnexal SFT generally follows a benign course, with a good prognosis after complete excision. Recurrence could occur many years after surgery which requiring careful and long-term follow-up.目的： 探讨眼附属器孤立性纤维性肿瘤（SFT）的临床病理学特点及预后。 方法： 回顾性病例系列研究。收集天津市眼科医院2000年1月至2020年12月收治的35例眼附属器SFT患者的临床和组织病理学资料，总结并分析患者的病史、影像学、组织病理学和免疫组织化学染色的特征、分型及治疗方法，并对患者进行随访。依照2013年世界卫生组织对SFT的诊断和分类标准，对所有患者进行病理学分类。 结果： 35例患者中，男性21例（60.0%）、女性14例（40.0%）；均为单眼发病，其中右眼23例（65.7%），左眼12例（34.3%）。年龄44（35，54）岁，范围为17~83岁。病程12（6，36）个月，范围为2个月至11年。临床表现包括眼球突出、眼球转动受限、复视及流泪等。肿瘤可发生于眼眶任何部位，以眶上部多见（19例，73.1%）。所有患者均手术完整切除肿物。影像学检查，肿瘤显示边界清楚的占位性病变，增强扫描后呈不均匀强化，肿瘤内含有丰富的血流信号。MRI检查显示加权成像T1WI呈等或稍低信号，加权成像T2WI信号显著增强，呈中高混杂信号。肿瘤长径2.1（1.5，2.6）cm。组织病理学分型：经典型23例（65.7%），巨细胞型2例（5.7%），黏液型8例（22.9%），恶性2例（5.7%）。免疫组织化学染色显示均对Vimentin、CD34、STAT6呈阳性表达；21例（60.0%）BCL-2呈阳性表达，Ki-67阳性指数为1.0%~10.0%。按照Demicco风险分层，35例患者均为低风险。术后获取随访资料25例，随访时间88（61，124）个月，范围为2年至14年7个月。2例复发，无患者出现远处转移或死亡。 结论： 眼附属器SFT主要临床表现为无痛缓慢生长的肿块，多数为经典型SFT，影像学表现多样。手术完整切除肿瘤预后较好，可于术后多年复发，需长期密切随访。.