Precision Transformation of Meningioma Diagnosis and Treatment: Advances in Molecular Pathology and Systemic Therapy

Meningiomas are the most common primary intracranial tumors of the central nervous system. They exhibit highly heterogeneous biological behavior that current histological grading cannot reliably predict. Currently, there are no standardized systemic treatment regimens for recurrent or refractory meningiomas; therefore, molecular subtyping is crucial for assessing prognosis and guiding treatment decisions. Key alterations, including NF2, TERT promoter, CDKN2A/B, AKT1, and SMO, contribute to the proliferation, invasion, and recurrence of meningiomas. This review summarizes the advances in molecular pathological alterations and clinical studies on systemic therapy for meningiomas. Anti-angiogenic agents, immune checkpoint inhibitors, and inhibitors targeting multiple signaling pathways represent promising directions. Anti-angiogenic therapy has demonstrated disease control, whereas PD-1 inhibitors have shown limited benefit in unstratified populations but may be of value in specific molecular subgroups. Molecularly stratified umbrella and basket trials are advancing precision treatment, and peptide receptor radionuclide therapy provides benefit for patients with specific molecular alterations. Some early trials of targeted therapies and immunotherapies have shown positive signals, but the overall level of evidence remains limited. Future studies should employ more rigorous trial designs and encourage patient participation to clarify the clinical value of each strategy and drive the shift from empirical to molecularly guided treatment.