Meningioma pathophysiology: Difference between revisions
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{{Meningioma}} | {{Meningioma}} | ||
{{CMG}} {{AE}}{{HL}} | {{CMG}} {{AE}} {{IO}} {{HL}} | ||
==Overview== | ==Overview== | ||
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. | 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 [[Gene|genes]] involved includes [[NF2 gene|NF2]], [[MEG3]], [[NDRG2]], and [[SMARCE1]]. [[Multiple endocrine neoplasia type 1|Multiple endocrine neoplasia 1]], [[cowden syndrome]], [[werner syndrome]] and [[Neurofibromatosis type II|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]]. | ||
==Pathogenesis== | ==Pathogenesis== | ||
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*On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma.<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015</ref> | *On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma.<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015</ref> | ||
[[File:Gross pathology meningioma.jpg |400px|thumb|left|Image showing gross pathology of a meningioma [https://radiopaedia.org/articles/meningioma?lang=us source:Case courtesy of Dr Dharam Ramnani, <a href="https://radiopaedia.org/">Radiopaedia.org</a>. ]]] | |||
[[File:Gross pathology meningioma.jpg|thumb| | <br style="clear:left" /> | ||
==Microscopic Pathology== | ==Microscopic Pathology== | ||
* On microscopic pathology, characteristic findings of meningioma include:<ref name="ComminsAtkinson2007">{{cite journal|last1=Commins|first1=Deborah L.|last2=Atkinson|first2=Roscoe D.|last3=Burnett|first3=Margaret E.|title=Review of meningioma histopathology|journal=Neurosurgical Focus|volume=23|issue=4|year=2007|pages=E3|issn=1092-0684|doi=10.3171/FOC-07/10/E3}}</ref><ref name="pmid25744347">{{cite journal| author=Shibuya M| title=Pathology and molecular genetics of meningioma: recent advances. | journal=Neurol Med Chir (Tokyo) | year= 2015 | volume= 55 | issue= 1 | pages= 14-27 | pmid=25744347 | doi=10.2176/nmc.ra.2014-0233 | pmc=4533397 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25744347 }} </ref> | * On microscopic pathology, characteristic findings of meningioma include:<ref name="ComminsAtkinson2007">{{cite journal|last1=Commins|first1=Deborah L.|last2=Atkinson|first2=Roscoe D.|last3=Burnett|first3=Margaret E.|title=Review of meningioma histopathology|journal=Neurosurgical Focus|volume=23|issue=4|year=2007|pages=E3|issn=1092-0684|doi=10.3171/FOC-07/10/E3}}</ref><ref name="pmid25744347">{{cite journal| author=Shibuya M| title=Pathology and molecular genetics of meningioma: recent advances. | journal=Neurol Med Chir (Tokyo) | year= 2015 | volume= 55 | issue= 1 | pages= 14-27 | pmid=25744347 | doi=10.2176/nmc.ra.2014-0233 | pmc=4533397 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25744347 }} </ref> | ||
:* Interdigitating processes and intercellular junctions | :* Interdigitating processes and intercellular junctions | ||
:* Small foci of necrosis surrounded by pseudopalisading tumor cells in nonembolized atypical meningiomas | :* 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 | :* [[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 | :* Prominent macronucleoli in the perinecrotic areas in embolized meningiomas | ||
:* Mitotic figures (low in benign cases and high in malignant and atypical cases) | :* 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 | :* 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 | :* Small cells with a high nuclear:cytoplasmic ratio | ||
:* Prominent nucleoli | :* Prominent [[nucleoli]] | ||
:* Uninterrupted patternless or sheet-like growth | :* Uninterrupted patternless or sheet-like growth | ||
:* Increased cellularity | :* Increased cellularity | ||
* The following immunohistochemistry profile can be used to support the diagnosis of meningioma:<ref name="ComminsAtkinson2007">{{cite journal|last1=Commins|first1=Deborah L.|last2=Atkinson|first2=Roscoe D.|last3=Burnett|first3=Margaret E.|title=Review of meningioma histopathology|journal=Neurosurgical Focus|volume=23|issue=4|year=2007|pages=E3|issn=1092-0684|doi=10.3171/FOC-07/10/E3}}</ref> | * The following immunohistochemistry profile can be used to support the diagnosis of meningioma:<ref name="ComminsAtkinson2007">{{cite journal|last1=Commins|first1=Deborah L.|last2=Atkinson|first2=Roscoe D.|last3=Burnett|first3=Margaret E.|title=Review of meningioma histopathology|journal=Neurosurgical Focus|volume=23|issue=4|year=2007|pages=E3|issn=1092-0684|doi=10.3171/FOC-07/10/E3}}</ref> | ||
:* Positive for vimentin | :* Positive for [[vimentin]] | ||
:* Negative for cytokeratin | :* Negative for [[cytokeratin]] | ||
:* Weak or negative staining for S 100 protein | :* Weak or negative staining for S 100 protein | ||
:* Focal membranous positivity for EMA | :* Focal membranous positivity for EMA | ||
[[File:Prominent mitosis meningioma.jpg|400px|thumb|left|Histology slide showing meningioma with prominent mitosis [https://commons.wikimedia.org/wiki/File:Prominent_mitosis_meningioma.jpg source:Jensflorian-wikimedia commons ]]] | |||
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[[File:Secretory meningioma 1.jpg|400px|thumb|left|Histology slide showing secretory meningioma [https://commons.wikimedia.org/wiki/File:Neuropathology_case_III_-_04.jpg source:Jensflorian-wikimedia commons ]]] | |||
<br style="clear:left" /> | |||
[[File:Meningioma histology.jpg|400px|thumb|left|Histology showing meningioma [https://radiopaedia.org/articles/meningioma?lang=us source:Case courtesy of Dr Dharam Ramnani, <a href="https://radiopaedia.org/">Radiopaedia.org</a>. ]]] | |||
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==References== | ==References== | ||
Latest revision as of 14:29, 10 September 2019
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Meningioma pathophysiology On the Web |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Odukwe, M.D. [2] Haytham Allaham, M.D. [3]
Overview
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.
Pathogenesis
- Meningiomas are the most common benign tumors of the brain. They are also the most common nonglial brain tumors.[1]
- 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.[2]
- 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.[1]
- The majority of meningiomas are benign (90%), about 6% are atypical, and 2% are malignant.[1]
- 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.[1]
- Meningiomas may possess receptors for platelet derived growth factor, vascular endothelial growth factor (VEGF), glucocorticoid, and epidermal growth factor.[1]
- 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%).[3]
- The symptoms of meningioma can be flared by water retention, engorgement of blood vessels, and the presence of sex hormone receptors on tumor cells.[1]
- 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.[3]
- 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.[3]
- The characterization of a meningioma being malignant is based on one or more of the following criteria: brain invasion, frank anaplasia, and distant metastasis[4]
Genetics
Genes involved in the pathogenesis of meningioma include:[5][4][6][7][8][9][10][11]
- 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.
- TRAF7
- AKT1
- KLF4
- SMO
- PIK3CA
Associated Conditions
Conditions associated with meningioma include:[12]
- 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
Gross Pathology
- On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma.[13]
Microscopic Pathology
- 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:[4]
- Positive for vimentin
- Negative for cytokeratin
- Weak or negative staining for S 100 protein
- Focal membranous positivity for EMA
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Gurcay AG, Bozkurt I, Senturk S, Kazanci A, Gurcan O, Turkoglu OF; et al. (2018). "Diagnosis, Treatment, and Management Strategy of Meningioma during Pregnancy". Asian J Neurosurg. 13 (1): 86–89. doi:10.4103/1793-5482.181115. PMC 5820904. PMID 29492130.
- ↑ Wiemels J, Wrensch M, Claus EB (2010) Epidemiology and etiology of meningioma. J Neurooncol 99 (3):307-14. DOI:10.1007/s11060-010-0386-3 PMID: 20821343
- ↑ 3.0 3.1 3.2 Sumkovski R, Micunovic M, Kocevski I, Ilievski B, Petrov I (2019). "Surgical Treatment of Meningiomas - Outcome Associated With Type of Resection, Recurrence, Karnofsky Performance Score, Mitotic Count". Open Access Maced J Med Sci. 7 (1): 56–64. doi:10.3889/oamjms.2018.503. PMC 6352459. PMID 30740161.
- ↑ 4.0 4.1 4.2 4.3 Commins, Deborah L.; Atkinson, Roscoe D.; Burnett, Margaret E. (2007). "Review of meningioma histopathology". Neurosurgical Focus. 23 (4): E3. doi:10.3171/FOC-07/10/E3. ISSN 1092-0684.
- ↑ Yuzawa S, Nishihara H, Tanaka S (2016). "Genetic landscape of meningioma". Brain Tumor Pathol. 33 (4): 237–247. doi:10.1007/s10014-016-0271-7. PMID 27624470.
- ↑ Balik V, Srovnal J, Sulla I, Kalita O, Foltanova T, Vaverka M; et al. (2013). "MEG3: a novel long noncoding potentially tumour-suppressing RNA in meningiomas". J Neurooncol. 112 (1): 1–8. doi:10.1007/s11060-012-1038-6. PMID 23307326.
- ↑ Lusis EA, Watson MA, Chicoine MR, Lyman M, Roerig P, Reifenberger G; et al. (2005). "Integrative genomic analysis identifies NDRG2 as a candidate tumor suppressor gene frequently inactivated in clinically aggressive meningioma". Cancer Res. 65 (16): 7121–6. doi:10.1158/0008-5472.CAN-05-0043. PMID 16103061.
- ↑ Skiriute D, Tamasauskas S, Asmoniene V, Saferis V, Skauminas K, Deltuva V; et al. (2011). "Tumor grade-related NDRG2 gene expression in primary and recurrent intracranial meningiomas". J Neurooncol. 102 (1): 89–94. doi:10.1007/s11060-010-0291-9. PMID 20607352.
- ↑ Smith MJ, O'Sullivan J, Bhaskar SS, Hadfield KD, Poke G, Caird J; et al. (2013). "Loss-of-function mutations in SMARCE1 cause an inherited disorder of multiple spinal meningiomas". Nat Genet. 45 (3): 295–8. doi:10.1038/ng.2552. PMID 23377182.
- ↑ van den Munckhof P, Christiaans I, Kenter SB, Baas F, Hulsebos TJ (2012). "Germline SMARCB1 mutation predisposes to multiple meningiomas and schwannomas with preferential location of cranial meningiomas at the falx cerebri". Neurogenetics. 13 (1): 1–7. doi:10.1007/s10048-011-0300-y. PMID 22038540.
- ↑ Goutagny S, Nault JC, Mallet M, Henin D, Rossi JZ, Kalamarides M (2014). "High incidence of activating TERT promoter mutations in meningiomas undergoing malignant progression". Brain Pathol. 24 (2): 184–9. doi:10.1111/bpa.12110. PMID 24261697.
- ↑ Kerr K, Qualmann K, Esquenazi Y, Hagan J, Kim DH (2018). "Familial Syndromes Involving Meningiomas Provide Mechanistic Insight Into Sporadic Disease". Neurosurgery. 83 (6): 1107–1118. doi:10.1093/neuros/nyy121. PMC 6235681. PMID 29660026.
- ↑ Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015
- ↑ Shibuya M (2015). "Pathology and molecular genetics of meningioma: recent advances". Neurol Med Chir (Tokyo). 55 (1): 14–27. doi:10.2176/nmc.ra.2014-0233. PMC 4533397. PMID 25744347.