眼眶孤立性纤维性肿瘤:单中心38例病例分析

Orbital solitary fibrous tumors:a single-institution study of 38 cases

目的 分析眼眶原发孤立性纤维性肿瘤(solitary fibrous tumors,SFT)的临床表现、手术方式选择及预后情况。设计回顾性病例系列。研究对象北京同仁医院2017-2022年经病理证实的眼眶原发性SFT患者38例。方法分析患者的病程、症状、体征、影像学和组织病理学以及随访和生存资料。应用Kaplan-Meier曲线分析无复发生存率(recurrence-free survival,RFS)和总生存率(overall survival,OS)。主要指标视力、症状及体征,病理学特征,复发和生存情况。结果初发病例的年龄中位数为37岁(5~65岁),复发病例的年龄中位数为64.5岁(41~81岁)(P=0.000)。术前影像学检查对SFT的灵敏度为22.58%,而误诊率为41.93%。术后病理学检查显示SFT肿瘤CD34阳性占97.37%,STAT6阳性占100%。有随访资料的37例患者平均病程(8.6±7.6)年(1.9~31.0年),其中16例复发病例平均病程(15.1±7.8)年(3.9~31.0年),复发病例第一次手术与最后一次手术平均间隔(12.7±8.0)年(1.3~29.7年)。36例有生存资料(排除活检和失访各1例)的患者术后(初发病例首次手术,复发病例末次手术)OS和RFS均为91.67%,平均随访时间分别为(2.2±1.4)年(0.1~5.8年)和(2.4±1.9)年(0.0~5.8年)。10例恶性SFT中,4例术后放疗,目前无复发;6例未放疗,其中1例复发死亡,5例目前无复发。3例复发,其中1例为恶性,2例为良性。3例死亡,其中2例为肿瘤相关性死亡。结论眼眶SFT初发病例多为年轻人。无论良恶性眼眶SFT均可复发,且易蔓延至颅内。良性SFT颅内广泛蔓延也可导致患者死亡。单纯影像学检查可能将SFT误诊为其他肿瘤。

Objective To analyze the clinical manifestations,surgical options and prognosis of primary solitary fibrous tumors(SFTs)of orbit.Design Retrospective cases series.Participants 38 cases of primary orbital SFTs confirmed by pathology from 2017 to 2022 in Beijing Tongren Hospital.Methods The course of the disease,symptoms,signs,imaging and histopathology,as well as follow-up and survival data were analyzed.And recurrence free survival(RFS)and overall survival(OS)were analyzed by Kaplan-Meier curve.Main Outcome Measures Visual acuity,clinical symptoms and signs,pathological features,recurrence,and survival.Results The median age of initial cases and recurrent cases was 37 years(5~65 years)and 64.5 years(41~81 years),respectively(P=0.000).The sensitivity of preoperative imaging examination for SFTs was 22.58%,and the misdiagnosis rate was 41.93%.Pathological examination showed that 97.37%of all SFTs tumors were CD34 positive,and all tumors were STAT6 positive(100%).The mean course of disease of 37 patients with follow-up data was 8.6±7.6 years(1.9~31.0 years),and the mean course of disease of 16 patients with recurrence was 15.1±7.8 years(3.9~31.0 years).The mean interval between the first operation and the last operation was 12.7±7.9 years(1.3~29.7 years).The 36 patients with survival data(excluding biopsy and one case of lost follow-up)had 91.67%OS and RFS after surgery(first operation for primary cases,last operation for recurrent cases),and the mean follow-up time was 2.3±1.4 years(0.1~5.8 years)and 2.4±1.9 years(0.0~5.8 years),respectively.Of the 10 cases of malignant SFTs,4 cases received postoperative radiotherapy and no recurrence was reported.6 cases did not receive radiotherapy,of which 1 case relapsed and died,and 5 cases had no relapse at present.3 cases recurred,of which 1 was malignant and 2 benign.Three cases died,of which two were tumor-related.Conclusion Most primary cases of orbital SFTs occur in young adults.Both benign and malignant orbital SFTs can recurs and spread to the intracranial easily.Extensive intracranial spread of benign SFTs can also lead to death.Imaging examination may misdiagnose SFTs as other tumors.