首页|102例中枢神经系统孤立性纤维性肿瘤的临床特点及预后相关因素分析

102例中枢神经系统孤立性纤维性肿瘤的临床特点及预后相关因素分析

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目的 探讨中枢神经系统孤立性纤维性肿瘤(SFT)患者的临床特点及预后的影响因素.方法 回顾性分析2016年1月至2020年12月首都医科大学附属北京天坛医院神经外科学中心手术治疗的102例SFT患者的临床资料.收集患者的临床资料、围手术期及术后复查头颅MRI检查结果、随访期生存情况和疾病进展情况,收集肿瘤的手术切除程度和病理学检查结果.采用寿命表法计算1、3、5年的无进展生存率和总生存率.采用log-rank检验分别比较不同年龄、肿瘤世界卫生组织(WHO)分级、手术切除程度、肿瘤最大径、肿瘤部位分组患者的生存分布,分别比较术前是否存在瘤周水肿、肿瘤是否不均匀强化、肿瘤是否累及静脉窦、肿瘤是否颅外侵袭以及是否行术后辅助放疗(PORT)患者的生存分布;进一步采用多因素Cox比例风险模型进行影响因素分析,确定无进展生存期及总生存期的影响因素.并采用log-rank检验比较不同治疗策略对SFT患者生存预后的影响.结果 102例患者的年龄为(45.9±13.4)岁,男女比例约为1.22:1.00.102例患者中,67例(65.7%)肿瘤位于小脑幕上,35例(34.3%)位于颅底;67例(65.7%)存在瘤周水肿,62例(60.8%)肿瘤不均匀强化,53例(52.0%)肿瘤累及静脉窦,9例(8.8%)肿瘤颅外侵袭.经病理学检查,肿瘤WHO 1级8例,2级49例,3级45例.102例患者中,肿瘤全切除(GTR)74例,次全切除(STR)28例;行PORT 57例.102例患者的随访时间[M(Q1,Q3)]为48(35,66)个月;至末次随访,30.4%(31/102)的患者肿瘤复发;9.8%(10/102)的患者因肿瘤进展死亡,1.0%(1/102)的患者死于术后并发症;4.9%(5/102)的患者肿瘤出现远处转移.102例患者1、3、5年的无进展生存率分别为80%、67%、67%;1、3、5年的总生存率分别为97%、91%、89%.单因素分析结果显示,肿瘤WHO分级较低、GTR、未累及静脉窦的患者无进展生存率较高(均P<0.05);年龄<60岁、肿瘤GTR的患者总生存率较高(均P<0.05).多因素Cox比例风险模型分析结果显示,肿瘤为WHO 3级(HR=4.45,95%CI:1.80-10.99,P<0.001)、STR(HR=9.82,95%CI:3.68~26.16,P<0.001)均是 SFT患者无进展生存期短的危险因素;而STR也是SFT患者总生存期短的危险因素(HR=8.73,95%CI:1.70-44.90,P=0.010).不同治疗策略分组患者的生存分析显示,总体队列中,与STR组(n=11)比较,STR+PORT组(n=17)患者的无进展生存率更高(P=0.026);肿瘤WHO分级为3级的患者中,与GTR组(n=5)比较,GTR+PORT组(n=23)患者的无进展生存率更高(P=0.029).结论 中枢神经系统SFT多见于小脑幕上,WHO 2、3级多见,可发生颅外转移,术后5年内的总体预后较好.肿瘤为WHO 3级及STR的患者无进展生存期较短,肿瘤STR的患者总生存期较短.
Analysis of clinical characteristics and prognostic factors of 102 cases of solitary fibrous tumors of the central nervous system
Objective To explore the clinical characteristics and prognostic factors of patients with solitary fibrous tumor(SFT)of the central nervous system.Methods The clinical data of 102 SFT patients who underwent surgical treatment at the Neurosurgery Center of Beijing Tiantan Hospital,Capital Medical University from January 2016 to December 2020 were retrospectively analyzed.The patients'clinical data,perioperative and postoperative brain MRI examination results,survival status and disease progression during the follow-up period,as well as the extent of tumor surgical resection and pathological examination results were collected.The life table method was used to calculate the progression-free survival rate and overall survival rate at 1,3,and 5 years.The log-rank test was used to compare the survival distribution of patients with different age,World Health Organization(WHO)tumor grade,surgical resection degree,maximum tumor diameter,and tumor location grouping,and to compare whether there was peritumoral edema before surgery and whether the tumor was unevenly enhanced after contrast administration,whether the tumor involved the venous sinus,whether the tumor invaded extracranially,and the survival distribution of patients with or without postoperative radiotherapy(PORT).Furthermore,a multivariate Cox proportional hazards model was used to conduct influencing factor analysis to determine the influencing factors of progression-free survival and overall survival.The log-rank test was used to compare the impact of different treatment strategies on the survival of SFT patients.Results The age of the 102 patients was 45.9±13.4 years old,and the male-to-female ratio was approximately 1.22:1.00.Among the 102 patients,67(65.7%)had tumors located on the supratentorial cerebellum,35(34.3%)located at the skull base;67 cases(65.7%)had peritumoral edema,62 cases(60.8%)had heterogeneous tumor contrast enhancement,53 cases(52.0%)involved venous sinuses,and 9 cases(8.8%)had extracranial invasion.After pathological examination,8 cases were WHO grade 1,49 cases were grade 2,and 45 cases were grade 3.Among the 102 patients,74 underwent total tumor resection(GTR),28 underwent subtotal resection(STR),and 57 underwent PORT.The median follow-up time of 102 patients was 48(35,66)months;by the last follow-up,30.4%(31/102)of patients had tumor recurrence;9.8%(10/102)died due to tumor progression,and 1.0%(1/102)died of postoperative complications;4.9%(5/102)patients developed distant metastasis.The progression-free survival rates of 102 patients at 1,3,and 5 years were 80%,67%,and 67%respectively;the overall survival rates at 1,3,and 5 years were 97%,91%,and 89%,respectively.The results of univariate analysis showed that patients with lower WHO grade,GTR,and tumors not involving venous sinuses had a higher progression-free survival rate(all P<0.05);patients aged<60 years and GTR had a higher overall survival rate(all P<0.05).Multivariate Cox proportional hazards model analysis results showed that the tumor WHO grade 3(HR=4.45,95%CI:1.80-10.99,P<0.001),STR(HR=9.82,95%CI:3.68-26.16,P<0.001)were all risk factors for short progression-free survival in SFT patients;and STR was also a risk factor for short overall survival in SFT patients(HR=8.73,95%CI:1.70-44.90,P=0.010).Survival analysis of patients grouped by different treatment strategies showed that in the overall cohort,compared with the STR group(n=11),the progression-free survival rate of patients in the STR+PORT group(n=17)was higher(P=0.026).Among patients with tumors of WHO grade 3,compared with the GTR group(n=5),the progression-free survival rate of patients in the GTR+PORT group(n=23)was higher(P=0.029).Conclusions SFT of the central nervous system is more common in the supratentorial cerebellum,and is more common in WHO grades 2 and 3.Extracranial metastasis can occur,and the overall prognosis within 5 years after surgery is good.Patients whose tumors are WHO grade 3 and STR have shorter progression-free survival,and patients with STR have shorter overall survival.

Central nervous system neoplasmsDisease attributesPrognosisTreatment outcomeSolitary fibrous tumor

王洪一、李达、王科、张力伟、张俊廷、吴震、王亮

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首都医科大学附属北京天坛医院神经外科学中心,北京 100070

中枢神经系统肿瘤 疾病特征 预后 治疗结果 孤立性纤维性肿瘤

2024

中华神经外科杂志
中华医学会

中华神经外科杂志

CSTPCD北大核心
影响因子:1.107
ISSN:1001-2346
年,卷(期):2024.40(6)