法国 2006-2015 年 399 例组织学证实的新诊断脑膜孤立性纤维瘤和血管外皮细胞瘤的描述性流行病学研究。
Descriptive epidemiology of 399 histologically confirmed newly diagnosed meningeal solitary fibrous tumours and haemangiopericytomas in France: 2006-2015.
机构信息
Department of Neurosurgery, Hôpital Privé Clairval - Ramsay Santé, 317 Bd de Redon, 13009, Marseille, France.
Department of Neurosurgery, Gui de Chauliac Hospital, Montpellier University Medical Center, 34295, Montpellier Cedex 5, France.
出版信息
Acta Neurochir (Wien). 2024 Aug 2;166(1):320. doi: 10.1007/s00701-024-06191-y.
PURPOSE
Meningeal solitary fibrous tumour (SFT) and haemangiopericytoma (HPC) are uncommon tumours that have been merged into a single entity in the last 2021 WHO Classification of Tumors of the Central Nervous System. To describe the epidemiology of SFT/HPC operated in France and, to assess their incidence.
METHODS
We processed the French Brain Tumour Database (FBTDB) to conduct a nationwide population-based study of all histopathologically confirmed SFT/HPC between 2006 and 2015.
RESULTS
Our study included 399 SFT/HPC patients, operated in France between 2006 and 2015, in one of the 46 participating neurosurgical centres. The incidence reached 0.062, CI[0.056-0.068] for 100,000 person-years. SFT accounted for 35.8% and, HPC for 64.2%. The ratio of SFT/HPC over meningioma operated during the same period was 0.013. SFT/HPC are about equally distributed in women and men (55.9% vs. 44.1%). For the whole population, mean age at surgery was 53.9 (SD ± 15.8) years. The incidence of SFT/HPC surgery increases with the age and, is maximal for the 50-55 years category. Benign SFT/HPC accounted for 65.16%, SFT/HPC of uncertain behaviour for 11.53% and malignant ones for 23.31%. The number of resection progresses as the histopathological behaviour became more aggressive. 6.7% of the patients with a benign SFT/HPC had a second surgery vs.16.6% in case of uncertain behaviour and, 28.4% for malignant SFT/HPC patients.
CONCLUSION
Meningeal SFT and HPC are rare CNS mesenchymal tumours which both share common epidemiological characteristics, asserting their merging under a common entity. SFT/HPC incidence is less that one case for 1 billion per year and, for around 100 meningiomas-like tumours removed, one SFT/HPC may be diagnosed. SFT/HPC are equally distributed in women and men and, are mainly diagnosed around 50-55 years. The more aggressive the tumour, the higher the probability of recurrence.
目的
脑脊膜孤立性纤维瘤（SFT）和血管外皮细胞瘤（HPC）是少见的肿瘤，在 2021 年世界卫生组织中枢神经系统肿瘤分类中已被合并为单一实体。本研究旨在描述法国脑脊膜 SFT/HPC 的流行病学特征，并评估其发病率。
方法
我们对法国脑肿瘤数据库（FBTDB）进行了处理，对 2006 年至 2015 年间所有经组织病理学证实的 SFT/HPC 进行了全国性的基于人群的研究。
结果
我们的研究纳入了 2006 年至 2015 年期间在法国 46 家参与神经外科中心之一接受手术治疗的 399 例 SFT/HPC 患者。发病率为 0.062，CI[0.056-0.068]每 100,000 人年。SFT 占 35.8%，HPC 占 64.2%。同期 SFT/HPC 与脑膜瘤的手术比例为 0.013。SFT/HPC 在男女中的分布大致相等（55.9% vs. 44.1%）。对于所有患者，手术时的平均年龄为 53.9（SD ± 15.8）岁。SFT/HPC 手术的发病率随年龄增长而增加，50-55 岁年龄段发病率最高。良性 SFT/HPC 占 65.16%，行为不确定的 SFT/HPC 占 11.53%，恶性 SFT/HPC 占 23.31%。随着组织病理学行为变得更具侵袭性，切除的数量也随之增加。6.7%的良性 SFT/HPC 患者需要再次手术，行为不确定的患者为 16.6%，恶性 SFT/HPC 患者为 28.4%。
结论
脑脊膜 SFT 和 HPC 是罕见的中枢神经系统间叶肿瘤，具有共同的流行病学特征，支持将它们合并为一个实体。SFT/HPC 的发病率为每年每 10 亿人不到 1 例，在大约 100 例脑膜瘤样肿瘤切除中，可能诊断出 1 例 SFT/HPC。SFT/HPC 在男女中的分布大致相等，主要在 50-55 岁左右诊断。肿瘤越具侵袭性，复发的可能性越高。
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