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Malignant (Anaplastic) Meningioma

An aggressive WHO grade 3 meningeal tumor with high recurrence and propensity for invasion.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Malignant (Anaplastic) Meningioma?

Malignant meningioma (anaplastic meningioma) is a WHO grade 3 meningeal tumor representing approximately 1-3% of all meningiomas. While the vast majority of meningiomas are benign (grade 1) or atypical (grade 2), grade 3 tumors are clinically aggressive with high recurrence rates and the potential for distant metastases (rare for meningiomas).

Histological criteria for grade 3 meningioma include features of frank malignancy resembling carcinoma, sarcoma, or melanoma, or a markedly elevated mitotic rate (20 or more per 10 high-power fields). The 2021 WHO classification incorporates molecular features such as TERT promoter mutations and CDKN2A/B homozygous deletion as criteria for grade 3 designation, even in tumors with lower mitotic rates.

Treatment requires multimodal management with maximal safe resection followed by adjuvant radiation. Despite aggressive treatment, recurrence is common and median survival is significantly shorter than for benign meningiomas. Systemic therapy options are limited but include hydroxyurea, somatostatin analogs, and emerging targeted therapies for specific molecular alterations. Repeated surgery and radiation are often required for recurrent disease.

Symptoms

Headache (often progressive)
Seizures (new onset or worsening)
Focal neurological deficits depending on location
Motor weakness or sensory loss
Visual disturbances (parasellar or olfactory groove)
Cognitive or personality changes (frontal location)
Cranial nerve palsies (skull base)
Cerebellar signs (posterior fossa)
Ataxia
Hearing loss (cerebellopontine angle)
Mass effect symptoms with rapid growth
Symptoms over weeks to months (faster than benign meningioma)
Recurrence symptoms after prior treatment
Local pain at surgical site (recurrence)

Risk Factors

Prior cranial radiation (highest known risk factor)
Neurofibromatosis type 2 (NF2)
Familial multiple meningioma syndromes
Schwannomatosis
Female sex (slight predominance, less than benign meningiomas)
Older age at presentation
Recurrence of lower-grade meningioma (progression to grade 3)
Hormonal factors (less established for malignant)
Most cases sporadic
Genetic alterations: TERT promoter, CDKN2A/B, NF2

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent headaches with neurological symptoms
  • First-time seizures in adulthood
  • Progressive focal neurological deficits
  • Cognitive changes
  • Visual disturbances or cranial nerve symptoms
  • Recurrence symptoms after prior meningioma treatment
  • History of cranial radiation with new symptoms
  • Family history of NF2 or hereditary meningiomas
  • Mass effect symptoms
  • Suspected raised intracranial pressure

Treatment Methods

01
Multidisciplinary neuro-oncology and neurosurgical evaluation
02
MRI brain with contrast, advanced imaging if needed
03
Maximal safe surgical resection (Simpson grade 1 ideal)
04
Histological grading and molecular profiling: TERT, CDKN2A/B, NF2
05
Adjuvant radiation therapy: standard for grade 3 (54-60 Gy)
06
Stereotactic radiosurgery: limited role for high-grade, considered for residual or small recurrence
07
Proton therapy: alternative for tumor location near critical structures
08
Recurrent disease: reoperation, reirradiation, salvage radiation
09
Systemic therapy options (limited evidence): hydroxyurea, somatostatin analogs (octreotide), bevacizumab
10
Targeted therapy: emerging — CDK4/6 inhibitors, mTOR inhibitors, FAK inhibitors (clinical trials)
11
Immunotherapy: checkpoint inhibitors in clinical trials
12
Anticonvulsant therapy: levetiracetam preferred
13
Corticosteroids: for symptomatic edema
14
Genetic counseling: NF2, familial meningioma syndromes
15
Long-term surveillance: MRI every 3-6 months initially, then less frequently
16
Multidisciplinary tumor board for treatment planning
17
Rehabilitation: physical, occupational, speech as needed
18
Palliative care integration
19
Clinical trial enrollment when possible
20
Psychosocial support given high recurrence risk

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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