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ENGLISH ABSTRACT
中枢神经系统孤立性纤维性肿瘤分级演变对临床病理学和预后分层的影响
李晓玲
张桦
胡承聪
周剑锋
庄梦艺
范欣欣
胡力文
陈余朋
黄茜
张声
王行富
作者及单位信息
·
DOI： 10.3760/cma.j.cn112152-20240810-00336
Evolution of grading for solitary fibrous tumors of the central nervous system: a clinical pathological and prognostic analysis
Li Xiaoling
Zhang Hua
Hu Chengcong
Zhou Jianfeng
Zhuang Mengyi
Fan Xinxin
Hu Liwen
Chen Yupeng
Huang Qian
Zhang Sheng
Wang Xingfu
Authors Info & Affiliations
Li Xiaoling
Department of Pathology, the Second Hospital of Longyan, Longyan 364000, China
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Zhang Hua
Department of Radiology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Radiology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
Hu Chengcong
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Pathology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
Zhou Jianfeng
Fujian Medical University, Basic Medical College, Five-Year Clinical Medicine Program, Fuzhou 350005, China
Zhuang Mengyi
Fujian Medical University, Basic Medical College, Five-Year Clinical Medicine Program, Fuzhou 350005, China
Fan Xinxin
Fujian Medical University, Basic Medical College, Five-Year Clinical Medicine Program, Fuzhou 350005, China
Hu Liwen
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Pathology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
Chen Yupeng
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Pathology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
Huang Qian
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Pathology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
Zhang Sheng
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Pathology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
Wang Xingfu
Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China
Department of Pathology, Binhai Campus of the First Affiliated Hospital, National Regional Medical Center, Fujian Medical University, Fuzhou 350212, China
·
DOI： 10.3760/cma.j.cn112152-20240810-00336
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摘要

目的探讨2021年WHO第5版中枢神经系统（CNS）肿瘤分类对孤立性纤维性肿瘤（SFT）的命名和分级体系进行了重大修订后，CNS SFT的分级变化及其与临床病理学特征和预后的关系。

方法回顾性分析2006年3月至2021年6月福建医科大学附属第一医院诊断为CNS SFT的患者（82例）的临床病理资料，根据WHO第5版CNS肿瘤分类标准对患者的病理进行重新分级，并对组织形态学、免疫组织化学特征以及临床影像资料进行综合分析。

结果患者年龄21～83岁，中位年龄48岁。82例患者完成了随访，死亡10例，复发24例，转移5例。MRI成像显示，SFT在T1加权像上呈等信号，T2加权像（T2WI）上信号复杂，且胶原纤维含量增加时信号强度降低。根据2021年WHO第5版CNS肿瘤分类的分级标准，SFT的分级发生了显著变化，1级SFT从2016年标准的10例增加至39例，而2级和3级相应减少。2016年分级系统与患者总生存时间（OS）有关（ P=0.009），而2021年分级系统则未达统计学意义。两版分级系统均与组织学表型、Ki-67指数、核分裂象以及坏死等有关（ P＜0.05）。所有患者均表达STAT6，波形蛋白、CD99、BCL-2及CD34不同程度表达。Ⅳ型胶原纤维的染色强度与患者OS有关（ P=0.017）。

结论CNS SFT新的分级系统变动大，其与OS的关联性尚需进一步验证。对SFT中胶原纤维的含量及其细微结构的深入研究，可能对患者的预后评估和治疗方案的制定具有重要的临床意义。通过定量分析T2WI信号强度，可能为术前初步评估SFT的胶原纤维含量提供一种新方法。

孤立性纤维性肿瘤;中枢神经系统;WHO分级;预后;胶原纤维
ABSTRACT

ObjectiveThe 5th edition of the WHO classification of central nervous system (CNS) tumors in 2021 made significant revisions to the nomenclature and grading system of solitary fibrous tumors (SFT). This study aimed to explore the changes in the grading of CNS SFT and its relationship with clinical pathological features and prognosis.

MethodsThis study retrospectively reviewed the clinical and pathological data of 82 patients with CNS SFT diagnosed at the First Affiliated Hospital of Fujian Medical University from March 2006 to June 2021, reassessed their grading according to the WHO 5th edition CNS tumor classification, and conducted a comprehensive analysis of their histological morphology, immunohistochemical characteristics, and clinical imaging data.

ResultsThe age of the patients ranged from 21 to 83 years, with a median age of 48 years. Follow-up was completed for 82 patients, during which 10 patients died, 24 recurred, and 5 metastasized. MRI imaging showed that SFT exhibited isointense signals on T1-weighted imaging (T1WI) and complex signals on T2-weighted imaging (T2WI), with signal intensity decreasing as the content of collagen fibers increased. According to the 2021 grading criteria, there was a significant change in the grading of SFT, with the number of grade 1 SFT increasing from 10 cases under the 2016 standard to 39 cases, while the number of grade 2 and 3 SFT decreased accordingly. The 2016 grading system was significantly correlated with the overall survival (OS) of patients ( P=0.009), while the 2021 grading system did not reach statistical significance. Both grading systems were correlated with histological phenotype, Ki-67 index, mitotic figures, and necrosis ( P＜0.05). All cases expressed STAT6, and showed varying degrees of expression of vimentin, CD99, BCL-2, and CD34. The staining intensity of type Ⅳ collagen fibers, as analyzed semi-quantitatively, was correlated with the OS of the patients ( P=0.017).

ConclusionsThe new grading system for CNS SFT has undergone significant changes, and its association with OS requires further validation. In-depth study of the content and fine structure of collagen fibers in SFT may have important clinical significance for the prognosis assessment and the formulation of treatment plans for patients. Moreover, quantitative analysis of T2WI signal intensity may provide a new method for preoperative preliminary assessment of the collagen fiber content in SFT.

Solitary fibrous tumor;Central nervous system;WHO grading;Prognosis;Collagen fiber
Wang Xingfu, Email: mocdef.6ab21ufxgnaw
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引用本文

李晓玲,张桦,胡承聪,等. 中枢神经系统孤立性纤维性肿瘤分级演变对临床病理学和预后分层的影响[J]. 中华肿瘤杂志,2025,47(03)：275-282.

DOI:10.3760/cma.j.cn112152-20240810-00336

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中枢神经系统（central nervous system，CNS）孤立性纤维性肿瘤（solitary fibrous tumor，SFT）在所有CNS原发肿瘤中的比例不足1%，通常见于40～60岁的成人 ［ 1 ］ 。手术切除联合放疗是CNS SFT的首选治疗方式，而靶向治疗是一种新兴的治疗手段。近年来，这类肿瘤的命名经历了一系列的更新和变化。2007年WHO在其神经系统肿瘤分类中将SFT和血管外皮细胞瘤（hemangiopericytoma，HPC）视为颅内2种独立的肿瘤实体 ［ 2 ］ 。然而，随着研究的深入，研究结果显示二者在临床表现、影像学特征以及病理学方面均具有一定的重叠，因此建议将它们归为同一肿瘤谱系 ［ 3 ］ 。2013年分子遗传学研究发现SFT和HPC具有相同的分子改变，即染色体12q13的倒置使得NAB2和STAT6基因融合，这一发现进一步支持了将二者视为同一肿瘤谱系的观点 ［ 4 , 5 ］ 。基于此，2016年WHO CNS肿瘤分类第4版修订版将SFT和HPC合并为SFT/HPC，并分为3级 ［ 6 ］ ，对应的国际疾病分类-肿瘤学编码分别为0、1和3。随着研究不断深入 ［ 1 ， 7 ］ ，2021年WHO CNS肿瘤分类去除了“HPC”这一术语，与软组织肿瘤的命名保持一致，将SFT/HPC统一命名为SFT，并继续分为3个等级 ［ 8 ］ ，国际疾病分类-肿瘤学编码为1，不同级别患者具有不同预后 ［ 9 ］ 。分级标准得到简化，主要依据核分裂象［5/10高倍镜视野（high power field，HPF）］及坏死这2个指标。通过简化分类体系、标准化命名、明确预后评估以及指导治疗决策，2021版分类为SFT患者的综合管理提供了更加科学、合理的依据。未来，随着对SFT分子机制研究的不断深入和新型疗法的不断涌现，患者的治疗效果和生存质量有望得到进一步提升。依据2021版CNS SFT分类回顾性分析福建医科大学附属第一医院收治的82例SFT患者的临床及病理资料，并复习相关文献。
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备注信息
A
王行富，Email： mocdef.6ab21ufxgnaw
B

李晓玲、张桦、胡承聪：数据收集、文献检索、论文撰写；李晓玲、黄茜、周剑锋、庄梦艺、范欣欣：数据整理、统计学分析、论文修改；王行富、张声：研究指导、论文修改；陈余朋、胡力文：免疫组化制作

C
李晓玲, 张桦, 胡承聪, 等. 中枢神经系统孤立性纤维性肿瘤分级演变对临床病理学和预后分层的影响[J]. 中华肿瘤杂志, 2025, 47(3): 275-282. DOI: 10.3760/cma.j.cn112152-20240810-00336.
D
所有作者声明无利益冲突
E
福建省卫生健康科技计划项目 (2021QNA073)
福建医科大学启航基金项目 (2021QH1090)
福建省大学生创新创业训练计划项目 (S202310392025)
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