Prevalence, natural history and surgical outcome of spinal meningiomas in NF2-related schwannomatosis.
AI interpretation is pending for this paper.
Open original publication →What the AI sees
Not AI summarized yet.
Research significance
Pending deeper interpretation.
Source 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.