Meningioma pathophysiology On the Web
American Roentgen Ray Society Images of Meningioma pathophysiology
Meningioma arises from the arachnoid "cap" cells, which are normally involved in the protection of the central nervous system by forming a thick envelope of meninges around the brain and spinal cord. The majority of meningiomas are benign. They can be found anywhere in the central nervous system but are most commonly seen in the parasagittal, convexity and turbeculum sellae areas. There may be genetic mutations involved in the development of a meningioma, some of the genes involved includes NF2, MEG3, NDRG2, and SMARCE1. Multiple endocrine neoplasia 1, cowden syndrome, werner syndrome and neurofibromatosis 2 are some of the conditions that may be associated with meningioma. On microscopic pathology, some of the characteristic findings of a meningioma include mitotic figures, necrosis, interdigitating processes, and brain invasion. Most meningiomas are positive for vimentin and negative for cytokeratin.
- Meningiomas are the most common benign tumors of the brain. They are also the most common nonglial brain tumors.
- Meningioma arises from the arachnoid "cap" cells, which are normally involved in the protection of the central nervous system by forming a thick envelope of meninges around the brain and spinal cord.
- Meningiomas are commonly found in the base of the skull and perivenous sinuses due to the abundance of arachnoid cap cells in these sites. They are usually non-infilterative.
- The majority of meningiomas are benign (90%), about 6% are atypical, and 2% are malignant.
- Some meningiomas may be positive for progesterone receptors on histological examination. This can lead to increased tumor size and symptom burden during pregnancy and the luteal phase of the menstural cycle.
- Meningiomas may possess receptors for platelet derived growth factor, vascular endothelial growth factor (VEGF), glucocorticoid, and epidermal growth factor.
- Meningiomas can be found anywhere in the central nervous system, with its most frequent distribution as follows: parasagittal (20.8%), then convexity (15.2%), and tuberculum sellae (12.8%).
- The symptoms of meningioma can be flared by water retention, engorgement of blood vessels, and the presence of sex hormone receptors on tumor cells.
- A meningioma can be localized in the following areas: sphenoid ridge, olfactory groove, falx, lateral ventriculi, tentorium, the middle fossa, the orbit, the spinal channel, the Sylvian fissure, extracalvarial, multiple localization, the pontocerebral angle, the sphenoidal plane, and the foramen magnum.
- The characteristics of a meningioma can be determined based on histopathological variables like tumor gradient, histological subtype, proliferative index, and invasiveness of a tumor to the brain.
- The characterization of a meningioma being malignant is based on one or more of the following criteria: brain invasion, frank anaplasia, and distant metastasis
- Neurofibromatosis 2 (NF2) gene on chromosome 22
- MEG3 (maternally expressed gene 3): Loss of expression, genomic DNA deletion, and promoter methylation on chromosome 14q32.
- NDRG2 (N-Myc downstream-regulated gene 2): Down regulation of this gene expression at the mRNA level is associated with the malignant progression and predisposition to recurrence of meningiomas.
- SMARCE1 (SWI/SNF chromatin-remodeling complex subunit gene): Heterozygous loss-of-function mutation. Its is commonly seen in meningiomas with clear cell histology and those located in the spine.
- SMARCB1: Predisposes to multiple meningiomas preferentially in the falx cerebri.
- TERT promoter mutation: Seen in meningiomas undergoing malignant histological progression.
Conditions associated with meningioma include:
- Neurofibromatosis type 2
- Nevoid basal cell carcinoma syndrome
- Multiple endocrine neoplasia 1 (MEN1)
- Cowden syndrome
- Werner syndrome
- BAP1 tumor predisposition syndrome
- Rubinstein-Taybi syndrome
- Familial meningiomatosis
- On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma.
- Interdigitating processes and intercellular junctions
- Small foci of necrosis surrounded by pseudopalisading tumor cells in nonembolized atypical meningiomas
- Necrosis occurring in large geographic areas with a quick line demarcating it from viable tissues in embolized menigiomas
- Prominent macronucleoli in the perinecrotic areas in embolized meningiomas
- Mitotic figures (low in benign cases and high in malignant and atypical cases)
- Brain invasion histologically seen as a finger-like, a tongue-like, or a knobby protrusion into the tissue
- Small cells with a high nuclear:cytoplasmic ratio
- Prominent nucleoli
- Uninterrupted patternless or sheet-like growth
- Increased cellularity
- The following immunohistochemistry profile can be used to support the diagnosis of meningioma:
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- Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015
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