Deboeuf Louise, Keraudy Antoine, Picart Thiebaud, Haddad Giorgio, Bernaoui Imen, Abi Jaoude Samiya, Aghakhani Nozar, Ducray François, Jacquesson Timothée, Meyronet David, Barrey Cédric Y, Jouanneau Emmanuel, Kalamarides Michel, Aboukais Rabih, Peyre Matthieu
Sorbonne Université - Department of Neurosurgery, Groupe Hospitalier Pitié-Salpêtrière, APHP, 47-83 boulevard de l'Hôpital, 75013, Paris, France.
Neurovascular Interfaces in Brain Tumors and Vascular Malformations - CRICM INSERM U1127 CNRS UMR 7225 - Brain Institute, Hôpital de la Pitié-Salpêtrière, Paris, France.
J Neurooncol. 2025 Sep 16. doi: 10.1007/s11060-025-05231-6.
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.
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.
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).
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.
脑膜瘤是与2型神经纤维瘤病相关的第二常见肿瘤类型。虽然颅内脑膜瘤的特征已得到广泛研究，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。
对在三个2型神经纤维瘤病参考中心接受SM手术切除的2型神经纤维瘤病患者进行了一项观察性回顾性研究。将数据与在皮提耶尔-萨尔佩特里埃医院接受散发性SM手术的一组患者进行比较。为了评估2型神经纤维瘤病中脊髓脑膜瘤的患病率，我们分析了在皮提耶尔-萨尔佩特里埃医院随访的2型神经纤维瘤病患者的颅脊髓MRI。
纳入了19例在1996年至2024年间接受29例SM手术的2型神经纤维瘤病患者（手术时的中位年龄：22岁；四分位间距：10 - 43岁）。最常见的部位是胸椎（22例，76%）。2型神经纤维瘤病患者比散发性SM患者明显年轻（32岁对64岁；p < 0.001），女性优势也不那么明显（2:1对9:1；p = 0.01）。散发性肿瘤患者的手术时间明显更短（6.3个月对36.5个月；p < 0.001）。两组在肿瘤位置、硬膜附着、分级、组织学亚型、完全切除率（85%对89%；p = 0.5）或复发风险（8%对9%；平均随访：7.5年；p = 0.9）方面未观察到显著差异。在115例至少有一次脑部和脊髓MRI检查的2型神经纤维瘤病患者中，39例（34%）有SM。与没有SM的2型神经纤维瘤病患者相比，有SM的患者颅内脑膜瘤的发生率更高（95%对64%；p < 0.001）。
脊髓脑膜瘤在2型神经纤维瘤病中很常见，大多与颅内脑膜瘤相关，并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，并未表现出更高的组织病理学侵袭性。
