Abstract

Simple SummaryThe most frequent intracranial neoplasm is meningioma. About 30% of these are represented by skull base meningiomas (SBMs). Patients with SBMs can be treated with a multimodal approach based on surgery, medical treatment and radiation-based therapy; however, the gold standard treatment for the majority of symptomatic meningiomas is still surgery. Surgical intervention is performed with the goal of maximum safe resection. This, however, poses technical challenges because of the proximity of these tumors with deep critical neurovascular structures, tumoral texture and consistency. A multimodal treatment, in combination with stereotactic radiosurgery and radiation therapy, is thus of utmost importance to achieve a satisfactory functional outcome and tumor control. The aim of this review was based on the identification of optimal multidisciplinary management of patients with SBMs. The investigation includes the relevant biological and clinical characteristics, the novel therapeutic approaches, highlighting the importance of a specialized multidisciplinary team, which is mandatory for SBM management.The surgical management of Skull Base Meningiomas (SBMs) has radically changed over the last two decades. Extensive surgery for patients with SBMs represents the mainstream treatment; however, it is often challenging due to narrow surgical corridors and proximity to critical neurovascular structures. Novel surgical technologies, including three-dimensional (3D) preoperative imaging, neuromonitoring, and surgical instruments, have gradually facilitated the surgical resectability of SBMs, reducing postoperative morbidity. Total removal is not always feasible considering a risky tumor location and invasion of surrounding structures and brain parenchyma. In recent years, the use of primary or adjuvant stereotactic radiosurgery (SRS) has progressively increased due to its safety and efficacy in the control of grade I and II meningiomas, especially for small to moderate size lesions. Patients with WHO grade SBMs receiving subtotal surgery can be monitored over time with surveillance imaging. Postoperative management remains highly controversial for grade II meningiomas, and depends on the presence of residual disease, with optional upfront adjuvant radiation therapy or close surveillance imaging in cases with total resection. Adjuvant radiation is strongly recommended in patients with grade III tumors. Although the currently available chemotherapy or targeted therapies available have a low efficacy, the molecular profiling of SBMs has shown genetic alterations that could be potentially targeted with novel tailored treatments. This multidisciplinary review provides an update on the advances in surgical technology, postoperative management and molecular profile of SBMs.

Highlights

  • Meningioma account for 16–36% of all intracranial tumors in adults [1]

  • Combination therapies with bevacizumab were tested, in a phase II study in which bevacizumab was combined with everolimus, an inhibitor of the mTOR pathway, in WorldHealth Organization (WHO) Grade I–III progressive/refractory meningiomas; this study demonstrated that the drug combination resulted in stable disease (SD) as the best response in 15 of 17 enrolled patients (88%) with 6 patients having a stable disease duration >12 months

  • This study demonstrated that 6m-progression-free survival (PFS) was found to be 64% and 37.5% in grade II and III meningiomas, respectively, with a median PFS of 6.5 for grade II meningiomas and 3.6 months for grade meningiomas, respectively

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Summary

Introduction

According to the World Health Organization, these lesions are currently classified into fifteen histotypes and three grades of malignancy, of which 90% are of Grade I [2]. The most significant prognostic factors for these tumors include the histological grade according to the World. Health Organization (WHO) criteria [2] and the extent of surgical resection according to the Simpson scale [3]. About 30% of intracranial meningiomas are represented by skull base meningiomas (SBMs) [4,5,6,7]. The surgical goal of radical resection is frequently hindered by the proximity of SBMs with deep critical neurovascular structures, complex vascularity, tumoral texture and consistency. The skull base was considered an inaccessible surgical location. Recent advances including the introduction of microsurgical techniques, improvements in imaging, virtual surgical simulation, and technological refinement of surgical instruments, along with the widespread use of minimally invasive approaches have radically changed SBM surgical management

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