Background: Intracranial malignant peripheral nerve sheath tumors are rare entities that carry a poor prognosis. To date, there are no established therapeutic strategies for these tumors.
Methods: We review the present treatment modalities and present the current therapeutic dilemmas. We perform a statistical analysis to evaluate the prognostic factors for Overall Survival of these patients. Additionally, we present our experience with a 64-year-old man with a MPNST of the left cerebellopontine angle.
Results: To our best knowledge, forty three patients with intracranial MPNSTs, including our case, have been published in the international literature. Our analysis showed gross total resection, radiotherapy and female gender to be beneficial prognostic factors of survival in the univariate analysis. Gross total resection was recognized as the only independent predictor of prolonged Overall Survival. In our case, we performed a gross total resection followed for the first time by stereotactically guided radiotherapy.
Conclusion: Considering the results of the statistical analysis and the known advantages of the stereotaxy, we suggest aggressive surgery followed by stereotactically guided radiotherapy as therapy of choice.