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Meningiomas

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Molecular Pathology of Nervous System Tumors

Part of the book series: Molecular Pathology Library ((MPLB,volume 8))

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Abstract

Meningiomas are the most frequent intracranial tumors. Meningiomas differ considerably by their histological appearance, the preferred site of tumor manifestation, biological behavior, recurrence rate, and overall survival. The most frequent alteration found in sporadic meningioma affects the NF2 gene. NF2 alterations are thought to represent the initial step for meningioma development and they are found in a considerable fraction of benign World Health Organization (WHO) grade I and atypical grade II/anaplastic grade III meningioma. Additional alterations in grade I meningioma affect the SMO, AKT1, KLF4, TRAF7, SMARCE1, and SMARCB1 genes, which are mostly exclusive of NF2 alterations. While NF2 is affected preferentially in fibroblastic/transitional grade I meningioma, KLF4/TRAF7 and SMARCE1 are detected nearly exclusively in secretory and clear-cell meningioma, respectively. Meningioma progression and recurrence is linked to inactivation of tumor suppressor genes at 1p, 9p, 10q, and 14q, and the CDKN2A/CDKN2B genes on chromosome 9p have been demonstrated to be exceptionally relevant. Other progression-associated changes are loss of progesterone receptor expression, inactivation of TIMP3, loss of DAL1/protein 4.1B, reduced expression of NDRG2 and MEG3, and increased telomerase activity. Genetically engineered mouse models of meningioma have lent additional support to the important tumor-initiating role of NF2 alterations, as well as to the role of CDKN2A/CDKN2B for malignant progression of meningioma.

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  1. Department of Neuropathology, Otto-von-Guericke University, Magdeburg, Germany

    Christian Mawrin M.D.

  2. Department of Neurosurgery, Hospital Pitie-Salpetriere, Paris, France

    Michel Kalamarides M.D., Ph.D.

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    Matthias A. Karajannis

  2. Professor of Pathology and Neurosurgery, NYU Langone Medical Center, New York, New York, USA

    David Zagzag

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Mawrin, C., Kalamarides, M. (2015). Meningiomas. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_17

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