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35例眼附属器孤立性纤维性肿瘤的临床病理分析

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

作者信息

Li J, Wang Y C, Chen L X, Lin J Y

机构信息

Tianjin Eye Hospital, Nankai University Affiliated Eye Hospital, Clinical College of Ophthalmology of Tianjin Medical University, Tianjin Eye Institute, Tianjin Key Laboratory of Ophthalmology and Visual Science, Tianjin 300020, China.

出版信息

Zhonghua Yan Ke Za Zhi. 2023 Mar 11;59(3):207-212. doi: 10.3760/cma.j.cn112142-20220407-00160.

Abstract

To analyze the clinical and pathological characteristics and prognosis of 35 cases of solitary fibrous tumor of ocular adnexal (SFT). 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. 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. 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.

摘要

分析35例眼附属器孤立性纤维瘤(SFT)的临床病理特征及预后。这是一项回顾性病例系列研究。收集了2000年1月至2020年12月在天津眼科医院就诊的35例眼附属器SFT患者的临床资料。分析患者的临床表现、影像学检查结果、病理特征、治疗情况,并进行随访。所有病例均按照2013年世界卫生组织软组织和骨肿瘤分类标准进行分类。男性21例(60.0%),女性14例(40.0%)。年龄范围为17至83岁,中位年龄为44(35,54)岁。所有患者均为单侧发病,右眼23例(65.7%),左眼12例(34.3%)。病程为2个月至11年,中位病程为12(6,36)个月。临床表现包括眼球突出、眼球运动受限、复视和流泪。所有患者均接受了肿瘤完全切除的手术治疗。眼附属器SFT多发生于眼眶上部(19例,73.1%)。影像学检查显示肿瘤边界清晰,为占位性病变,增强扫描呈不均匀强化,肿瘤内血流信号丰富。MRI表现为T1WI等信号或低信号,T2WI明显强化,呈中高不均匀信号。肿瘤直径为2.1(1.5,2.6)cm。经典型23例(65.7%),巨细胞型2例(5.7%),黏液样型8例(22.9%),恶性2例(5.7%)。免疫组化染色显示,所有患者波形蛋白、CD34和信号转导子和转录激活子6均呈阳性表达。21例(60.0%)BCL-2呈阳性表达,Ki-67阳性指数为1.0%至10.0%。根据Demicco风险分层,该组肿瘤均为低风险。对25例患者进行了随访,随访时间为2年至14年7个月,中位随访时间为88(61,124)个月。2例患者复发,未观察到远处转移或死亡。眼附属器SFT主要表现为无痛性、生长缓慢的肿块。大多数为典型的SFT。影像学表现多样。眼附属器SFT一般病程良性,完整切除后预后良好。术后多年可能复发,需要仔细和长期随访。

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