Tumors of Meningothelial Cells: Meningiomas
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Meningiomas are usually benign tumors arising from meningeal or arachnoithelial cells. They occur frequently and make up 40–45% of tumors seen in the daily clinical practice. According to biologic behavior, meningioma (with various histologic subtypes) (WHO grade I), atypical meningioma (WHO grade II), and anaplastic meningioma (WHO grade III) are distinguished.
Radiologically, meningiomas present as extra-axial, well-defined mass, attached to the dura with a round, lobulated, or en plaque configuration. Tumor grading on the basis of imaging findings is in the majority of the cases impossible.
Histologically, meningiomas (WHO grade I) are made up of tumor cells with uniform size, oval nuclei, delicate chromatin, and resemblance of arachnoid cap cells. Tumor cells form a syncytium with undiscernible cell processes. Atypical meningiomas (WHO grade II) show increased mitotic activity, brain invasion on histology, or at least increased cellularity, small cells with a high nuclear-to-cytoplasmic ratio, prominent nucleoli, sheeting (i.e., uninterrupted patternless or sheet-like growth), and foci of spontaneous (i.e., not iatrogenically induced) necrosis. Anaplastic (malignant) meningioma (WHO grade III) shows malignant cytology and/or markedly elevated mitotic activity.
Molecular changes affect the hedgehog (Hh) and the Wnt (“wingless”) pathways as well as the RB (retinoblastoma)/TP53 (tumor protein 53), the PI3K (phosphatidylinositol 3-kinase)/Akt, the MAPK (mitogen-activated protein kinase), and the Notch signaling pathways. Chromosomal aberrations and mutations are common. As epigenetic changes DNA methylation shows promoter hypermethylation of several tumor suppressor genes (e.g., TIMP-3, MEG3, NDRG2, and HOXA7, 9 and 10). Distinct expression patterns of genes of the Hh and Wnt pathways were described.
Treatment consists of surgery. Radiation is applied in high-grade tumors. Outcome is usually good.
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