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

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.