Epidemiology, Diagnosis, Histopathological Grading, Key Molecular Features

Chapter 10 - Classification and Treatment Strategies of Meningiomas

Epidemiology Diagnosis Figure 1

Courtesy J. Furtner, Medical University of Vienna, Vienna

Meningiomas are tumours derived from arachnoidal cells and are typically attached to the inner surface of the dura mater. Diagnosis is usually done by cranial magnetic resonance imaging (MRI).

Meningiomas represent the most common primary intracranial/intraspinal tumours in adults. Meninigiomas preferentially affect women. Paediatric meningiomas are exceptionally rare.

Risk factors include higher age, female gender, prior cranial irradiation and hereditary tumour syndromes such as neurofibromatosis Type 2 (NF2).

Common intracranial locations are the cerebral convexities, olfactory grooves, sphenoid wings, tentorium and posterior fossa. Spinal meningiomas are less common and are mainly found in the thoracic region.

Histopathology distinguishes 9 benign WHO Grade 1 forms, with meningothelial and broblastic/transitional meningiomas covering the majority of these tumours. 

About 20% represent aggressive Grade 2 meningiomas, while anaplastic Grade 3 tumours are rare, with 1%–5% of all tumours.

About 50% of meningiomas have molecular alterations in the NF2 gene. In NF2 wildtype tumours, mutations in Smo, Akt, or KLF4/TRAF7 genes occur in a small number of tumours. Patients suffering from germline NF2 alterations are prone to develop (multiple) meningiomas.

Revision Questions

  1. What are the main risk factors for meningioma development?
  2. Where do meningiomas preferentially grow?
  3. Are the majority of meningiomas benign or malignant?

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Last update: 18 September 2017