Prevalence, natural history and surgical outcome of spinal meningiomas in NF2-related schwannomatosis.

IF 3.1 2区医学 Q2 CLINICAL NEUROLOGY

Journal of Neuro-Oncology Pub Date : 2025-12-01 Epub Date: 2025-09-16 DOI:10.1007/s11060-025-05231-6

Louise Deboeuf, Antoine Keraudy, Thiebaud Picart, Giorgio Haddad, Imen Bernaoui, Samiya Abi Jaoude, Nozar Aghakhani, François Ducray, Timothée Jacquesson, David Meyronet, Cédric Y Barrey, Emmanuel Jouanneau, Michel Kalamarides, Rabih Aboukais, Matthieu Peyre

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引用次数: 0

Abstract

Purpose: Meningiomas are the second most common type of tumor associated with Neurofibromatosis Type 2 Schwannomatosis (NF2-SWN). While the characteristics of intracranial meningiomas have been extensively studied, data on the epidemiological and histological features of spinal meningiomas (SM) remain limited.

Methods: An observational, retrospective study was conducted on NF2-SWN patients who underwent surgical resection of SMs at three NF2-SWN reference centers. Data were compared to a cohort of patients operated on for sporadic SM at Pitié-Salpêtrière Hospital. To evaluate the prevalence of spinal meningiomas in NF2-SWN, we analyzed the craniospinal MRIs of NF2-SWN patients followed at the Pitié-Salpêtrière Hospital.

Results: Nineteen NF2-SWN patients who underwent surgery for 29 SMs between 1996 and 2024 were included (median age at surgery: 22 years; IQR:10-43). The most common location was the thoracic spine (22 cases, 76%). NF2-SWN patients were significantly younger than those with sporadic SMs (32 vs. 64 years; p < 0.001), and the female predominance was less pronounced (2:1 vs. 9:1; p = 0.01). Patients with sporadic tumors had a significantly shorter time to surgery (6.3 vs. 36.5 months; p < 0.001). No significant differences were observed between the two groups regarding tumor location, dural attachment, grade, histological subtype, rate of complete resection (85% vs. 89%; p = 0.5), or recurrence risk (8% vs. 9%; mean follow-up: 7.5 years; p = 0.9). Among the 115 NF2-SWN patients with at least one brain and spinal MRI, 39 (34%) had a SM. Patients with SM more frequently harbored intracranial meningiomas (95%) compared to NF2-SWN patients without SM (64%; p < 0.001).

Conclusion: Spinal meningiomas are common in NF2-SWN, mostly found in association with intracranial meningiomas and correlate with a higher tumor burden and more severe disease phenotype. However, these tumors progress slowly, rarely require surgical intervention, and do not demonstrate higher histopathological aggressiveness compared to sporadic spinal meningiomas.

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nf2相关神经鞘瘤病中脊髓脑膜瘤的患病率、自然病史和手术结果。

目的：脑膜瘤是与2型神经纤维瘤病（NF2-SWN）相关的第二大常见肿瘤。虽然颅内脑膜瘤的特征已经被广泛研究，但关于脊髓脑膜瘤（SM）的流行病学和组织学特征的数据仍然有限。方法：对在三个NF2-SWN参比中心接受手术切除SMs的NF2-SWN患者进行观察性回顾性研究。数据与在Pitié-Salpêtrière医院接受散发性SM手术的患者队列进行比较。为了评估NF2-SWN中脊髓脑膜瘤的患病率，我们分析了在Pitié-Salpêtrière医院随访的NF2-SWN患者的颅脊髓mri。结果：纳入了1996年至2024年间接受29个SMs手术的19例NF2-SWN患者（手术时中位年龄：22岁；IQR:10-43）。最常见的部位是胸椎（22例，76%）。结论：脊髓脑膜瘤在NF2-SWN患者中很常见，主要与颅内脑膜瘤相关，且与更高的肿瘤负荷和更严重的疾病表型相关。然而，这些肿瘤进展缓慢，很少需要手术干预，与散发性脊髓脑膜瘤相比，没有更高的组织病理学侵袭性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Neuro-Oncology 医学-临床神经学

CiteScore

6.60

自引率

7.70%

发文量

277

审稿时长

3.3 months

期刊介绍： The Journal of Neuro-Oncology is a multi-disciplinary journal encompassing basic, applied, and clinical investigations in all research areas as they relate to cancer and the central nervous system. It provides a single forum for communication among neurologists, neurosurgeons, radiotherapists, medical oncologists, neuropathologists, neurodiagnosticians, and laboratory-based oncologists conducting relevant research. The Journal of Neuro-Oncology does not seek to isolate the field, but rather to focus the efforts of many disciplines in one publication through a format which pulls together these diverse interests. More than any other field of oncology, cancer of the central nervous system requires multi-disciplinary approaches. To alleviate having to scan dozens of journals of cell biology, pathology, laboratory and clinical endeavours, JNO is a periodical in which current, high-quality, relevant research in all aspects of neuro-oncology may be found.