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PubMed This is a summary of 14 peer-reviewed journal articles Updated
Neuro-oncology

High-Grade and Recurrent Meningiomas: Emerging Therapies

At a Glance

For high-grade (WHO Grade 3) or recurrent meningiomas that no longer respond to surgery and radiation, treatment often shifts to systemic therapies. Options include targeted drugs, PRRT, and immunotherapies, which are selected based on molecular profiling of the tumor's specific DNA mutations.

A Note for Newly Diagnosed Patients: If you have recently been diagnosed with a meningioma, please know that the vast majority of these tumors are benign (WHO Grade 1) and will never require the aggressive treatments listed below. The following information applies only to a small minority of patients facing high-grade or recurrent tumors. Reading this section may cause unnecessary anxiety if it does not apply to your specific situation.

For patients with high-grade (WHO Grade 3) or recurrent meningiomas, the journey often moves beyond surgery and radiation. When these standard treatments are no longer enough, doctors look toward systemic therapies—medications that travel through the bloodstream to find and attack tumor cells. While there is currently no “gold standard” systemic treatment for meningioma, this is the most active frontier of clinical research [1][2].

Systemic Therapies: The Current Toolkit

When surgery and radiation have been exhausted, several types of medications may be considered, often under the guidance of clinical guidelines.

  • Anti-Angiogenesis Therapy (Bevacizumab): This medication works by cutting off the tumor’s blood supply by targeting VEGF, the protein that makes blood vessels leaky [3]. Some patients experience a “stabilization” of their disease [4][1]. Side Effects: It can cause high blood pressure, fatigue, and issues with wound healing or bleeding, which require close monitoring.
  • Somatostatin Analogs and PRRT: Many meningiomas have receptors for a hormone called somatostatin. Peptide Receptor Radionuclide Therapy (PRRT), specifically 177Lu-DOTATATE, attaches a radioactive molecule to a somatostatin-seeking drug, delivering radiation directly to the tumor cells from the inside [5][6]. Side Effects: Patients may experience fatigue, nausea, and temporary drops in blood cell counts (like white blood cells or platelets).
  • Targeted Therapies: If a tumor has a specific genetic mutation, targeted drugs may be used. For example, tumors with AKT1 or SMO mutations may respond to drugs designed to block those specific pathways [7][8].
  • Immunotherapy: Drugs like Nivolumab or Pembrolizumab (PD-1 inhibitors) are designed to “unmask” tumor cells so the body’s own immune system can attack them [9]. Research into these agents for high-grade meningiomas is ongoing [10].

The Importance of Molecular Profiling

Because recurrent and high-grade meningiomas are biologically complex, molecular profiling (or Next-Generation Sequencing) has become a vital tool. By analyzing the tumor’s DNA, doctors can identify:

  • NF2 Mutations: Often seen in more aggressive cases and may point toward certain targeted trials [11].
  • CDKN2A/B Deletions: A marker for high-grade behavior that helps doctors understand the need for more intensive management [12].
  • SSTR Expression: Testing for somatostatin receptors (often via a DOTATATE PET scan) to see if PRRT is a viable option [13].

Facing Recurrence

Dealing with a tumor that returns is an immense emotional and physical burden. It is important to work with a multidisciplinary team—including neuro-oncologists, radiation oncologists, and social workers—who can help you navigate these complex choices while prioritizing your well-being. Focusing on “disease control” rather than “cure” can sometimes help in setting realistic goals and maintaining a high quality of life [14].

Return to Home | Previous: Surgery & Radiation | Next: Survivorship & Monitoring

Common questions in this guide

What systemic therapies are used for recurrent meningiomas?
When surgery and radiation are no longer effective, doctors may use systemic medications. These include anti-angiogenesis drugs like Bevacizumab, targeted therapies for specific genetic mutations, immunotherapy, or targeted radiation like PRRT.
Why is molecular profiling important for high-grade meningiomas?
Molecular profiling, or next-generation sequencing, analyzes your tumor's DNA for specific mutations like NF2 or AKT1. Identifying these unique genetic markers helps doctors determine if you are a candidate for targeted therapies or specific clinical trials.
What is PRRT and how does it treat meningioma?
Peptide Receptor Radionuclide Therapy (PRRT) is a treatment that attaches a radioactive molecule to a drug that seeks out somatostatin receptors on tumor cells. It delivers targeted radiation directly to the meningioma from the inside.
Are most meningiomas high-grade and aggressive?
No, the vast majority of newly diagnosed meningiomas are benign (WHO Grade 1) and do not require aggressive systemic treatments. High-grade and recurrent meningiomas represent a small minority of cases.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Has my tumor undergone comprehensive genomic profiling (NGS) to look for mutations like AKT1, SMO, or NF2?
  2. 2.Given my tumor's recurrence, am I a candidate for a DOTATATE PET scan to see if PRRT is an option?
  3. 3.Are there any open clinical trials for high-grade meningioma that I should consider before we start the next round of standard treatment?
  4. 4.What is your experience using Bevacizumab for meningioma, and what side effects should I be most concerned about?
  5. 5.Does my tumor express PD-L1 or have a high tumor mutational burden (TMB) that would make immunotherapy a possibility?

Questions For You

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References

References (14)
  1. 1

    Chemotherapy and targeted therapies for meningiomas: what is the evidence?

    Graillon T, Tabouret E, Chinot O

    Current opinion in neurology 2021; (34(6)):857-867 doi:10.1097/WCO.0000000000001002.

    PMID: 34629433
  2. 2

    Multidisciplinary approach to anaplastic and metastatic meningioma: A case report and review of the literature.

    Ajler P, Davila EZG, Plou P, et al.

    Surgical neurology international 2023; (14()):230 doi:10.25259/SNI_222_2023.

    PMID: 37560559
  3. 3

    Systemic therapy for relapsed/refractory meningioma: Is there potential for antiangiogenic agents?

    Dasanu CA, Samara Y, Codreanu I, et al.

    Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners 2019; (25(3)):638-647 doi:10.1177/1078155218799850.

    PMID: 30253729
  4. 4

    A Retrospective Study on Prognostic Factors and Systemic Treatments of Refractory Meningiomas.

    Nguyen DC, Nader C, Bélanger K, et al.

    Current oncology (Toronto, Ont.) 2025; (32(9)) doi:10.3390/curroncol32090516.

    PMID: 41002586
  5. 5

    Two Birds with One Stone: Skull Base Meningioma and Jugulotympanic Paragangliomas with Somatostatin Receptor Positron Emission Tomography.

    Treglia G, Raditchkova M, Giovanella L, et al.

    Diagnostics (Basel, Switzerland) 2021; (11(9)) doi:10.3390/diagnostics11091669.

    PMID: 34574010
  6. 6

    Peptide Receptor Radionuclide Therapy in Patients with Advanced, Recurrent or Progressive Meningioma: An Updated Systematic Review and Meta-Analysis.

    Muoio B, Iacovitti CM, Bosetti DG, et al.

    Cancers 2025; (17(12)) doi:10.3390/cancers17122039.

    PMID: 40563688
  7. 7

    Systemic therapy for recurrent meningioma.

    Le Rhun E, Taillibert S, Chamberlain MC

    Expert review of neurotherapeutics 2016; (16(8)):889-901 doi:10.1080/14737175.2016.1184087.

    PMID: 27123883
  8. 8

    A Practical Overview on the Molecular Biology of Meningioma.

    Delgado-López PD, Cubo-Delgado E, González-Bernal JJ, Martín-Alonso J

    Current neurology and neuroscience reports 2020; (20(12)):62 doi:10.1007/s11910-020-01084-w.

    PMID: 33136206
  9. 9

    A Prospective, Cohort Study of SITOIGANAP to Treat Glioblastoma When Given in Combination With Granulocyte-Macrophage Colony-Stimulating Factor/Cyclophosphamide/Bevacizumab/Nivolumab or Granulocyte-Macrophage Colony-Stimulating Factor/Cyclophosphamide/Bevacizumab/Pembrolizumab in Patients Who Failed Prior Treatment With Surgical Resection, Radiation, and Temozolomide.

    Bota DA, Taylor TH, Lomeli N, et al.

    Frontiers in oncology 2022; (12()):934638 doi:10.3389/fonc.2022.934638.

    PMID: 35837107
  10. 10

    Phase 2 study of pembrolizumab in patients with recurrent and residual high-grade meningiomas.

    Brastianos PK, Kim AE, Giobbie-Hurder A, et al.

    Nature communications 2022; (13(1)):1325 doi:10.1038/s41467-022-29052-7.

    PMID: 35289329
  11. 11

    Recent advances in the molecular prognostication of meningiomas.

    Wang EJ, Haddad AF, Young JS, et al.

    Frontiers in oncology 2022; (12()):910199 doi:10.3389/fonc.2022.910199.

    PMID: 36686824
  12. 12

    Meningioma: Molecular Updates from the 2021 World Health Organization Classification of CNS Tumors and Imaging Correlates.

    Soni N, Ora M, Bathla G, et al.

    AJNR. American journal of neuroradiology 2025; (46(2)):240-250 doi:10.3174/ajnr.A8368.

    PMID: 38844366
  13. 13

    Theranostics in Neuroendocrine Tumors.

    Mallak N, O'Brien SR, Pryma DA, Mittra E

    Cancer journal (Sudbury, Mass.) 2024; (30(3)):185-193 doi:10.1097/PPO.0000000000000723.

    PMID: 38753753
  14. 14

    Preoperative risk factors associated with new focal neurological deficit and other major adverse events in first-time intracranial meningioma neurosurgery.

    Jenkins FS, Vasella F, Padevit L, et al.

    Acta neurochirurgica 2021; (163(10)):2871-2879 doi:10.1007/s00701-021-04897-x.

    PMID: 34259901

This page provides information on treatments for high-grade and recurrent meningiomas for educational purposes. Always consult your neuro-oncology team regarding your specific tumor profile and treatment plan.

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