Clinical course and management of intracranial meningiomas in neurofibromatosis type 2 patients

Abstract

Objective

The aim of this study is to evaluate our surgical experience with intracranial meningiomas in NF2 patients and provide knowledge of the natural history of these lesions.

Methods

We included in the natural growth study patients with the diagnosis of NF2 who harbored intracranial meningiomas and were observed for at least 1 year. Tumors that were resected before achieving long-term follow-up were excluded from this analysis.

Results

We found 118 intracranial meningiomas in 34 patients in our series. 8 meningiomas in 7 patients were symptomatic. It was found that with an increase in tumor volume, brain edema and with the tumor location at the skull base, meningiomas are more likely to be symptomatic. Univariate analysis revealed that tumor growth was associated with a younger age at the onset of NF2-related symptoms, greater initial tumor volume, brain edema and with the presence of intracranial non-vestibular schwannoma. Multivariate analysis showed that the probability of tumor growth is associated with prolonged follow-up time. De novo meningiomas exhibited a significantly higher growth rate than other meningiomas. These tumors were more frequent in patients with intracranial non-vestibular schwannoma and with increasing length of meningioma observation.

Conclusion

Meningiomas occur in about half NF2 patients. Many of them exhibit slow growth and long remain asymptomatic, however, those associated with early onset of NF2 symptoms and other features of the disease severity should be monitored in case of clinical and radiological progression that may require surgical treatment.