Meningioma pathophysiology: Difference between revisions

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{{CMG}} {{AE}}{{HL}}
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==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]].<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25th 2015</ref><ref name="C">Meningioma. Canadian Cancer Society http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/brain-and-spinal-tumours/meningioma/?region=mb September, 25th 2015</ref><ref name="radio">Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25th 2015</ref> The majority of meningiomas are [[benign]]. Development of meningioma is the result of multiple genetic mutations The majority of meningioma cases involve a deletion of the neurofibromatosis 2 gene located on [[chromosome 22]]. On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma. On microscopic [[histopathological]] analysis, whorled appearance, [[calcification]], and [[psammoma]] bodies are characteristic findings of meningioma.<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25th 2015</ref>
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]].<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015</ref><ref name="C">Meningioma. Canadian Cancer Society http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/brain-and-spinal-tumours/meningioma/?region=mb September, 25 2015</ref><ref name="radio">Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25 2015</ref> The majority of meningiomas are [[benign]]. Development of meningioma is the result of multiple genetic mutations The majority of meningioma cases involve a deletion of the neurofibromatosis 2 gene located on [[chromosome 22]]. On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma. On microscopic [[histopathological]] analysis, whorled appearance, [[calcification]], and [[psammoma]] bodies are characteristic findings of meningioma.<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25 2015</ref>


==Pathogenesis==
==Pathogenesis==
*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]].<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25th 2015</ref>  
*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]].<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015</ref>  
*The majority of meningiomas are [[benign]]; however, a minority of cases may develop a [[malignant]] nature.
*The majority of meningiomas are [[benign]]; however, a minority of cases may develop a [[malignant]] nature.
*The exact pathophysiology of meningioma depends on the histological subtype of the tumor.
*The exact pathophysiology of meningioma depends on the histological subtype of the tumor.
*Meningioma may be located either intradural or extradural along the central nervous system.<ref name="L">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview Accessed on September, 25th 2015</ref>
*Meningioma may be located either intradural or extradural along the central nervous system.<ref name="L">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview Accessed on September, 25 2015</ref>
*Meningiomas are frequently found attached to the [[dura]] surrounding the brain tissue in several locations that include:<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25th 2015</ref>
*Meningiomas are frequently found attached to the [[dura]] surrounding the brain tissue in several locations that include:<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015</ref>
:* Frontaparietal region
:* Frontaparietal region
:* [[Brain]] convexity  
:* [[Brain]] convexity  
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:* [[Confluence of sinuses]]
:* [[Confluence of sinuses]]
:* Parafalcine region
:* Parafalcine region
*Meningiomas are commonly associated with [[neurofibromatosis type 2]] syndrome and [[Gorlin syndrome]].<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25th 2015</ref>
*Meningiomas are commonly associated with [[neurofibromatosis type 2]] syndrome and [[Gorlin syndrome]].<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25 2015</ref>
*Meningiomas demonstrate [[estrogen]] and [[progesterone]] sensitivity and may grow during pregnancy.<ref name="radio">Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25th 2015</ref>
*Meningiomas demonstrate [[estrogen]] and [[progesterone]] sensitivity and may grow during pregnancy.<ref name="radio">Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25 2015</ref>


==Genetics==
==Genetics==
*Development of meningioma is the result of multiple [[genetic mutations]].<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25th 2015</ref>
*Development of meningioma is the result of multiple [[genetic mutations]].<ref name="wiki">Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015</ref>
*The majority of meningioma cases involve a deletion of the neurofibromatosis 2 gene located on [[chromosome 22]].
*The majority of meningioma cases involve a deletion of the neurofibromatosis 2 gene located on [[chromosome 22]].
*Other genes involved in the pathogenesis of meningioma include:
*Other genes involved in the pathogenesis of meningioma include:
Line 35: Line 35:


==Gross Pathology==
==Gross Pathology==
*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, 25th 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>




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==Microscopic Pathology==
==Microscopic Pathology==
*On microscopic [[histopathological]] analysis, whorled appearance, [[calcification]], and [[psammoma]] bodies are characteristic findings of of meningioma.<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25th 2015</ref>
*On microscopic [[histopathological]] analysis, whorled appearance, [[calcification]], and [[psammoma]] bodies are characteristic findings of of meningioma.<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25 2015</ref>
*The table below differentiates between the three main groups of meningioma according to WHO histological classification:<ref name="C">Meningioma. Canadian Cancer Society http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/brain-and-spinal-tumours/meningioma/?region=mb September, 25th 2015</ref><ref name="radio">Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25th 2015</ref><ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25th 2015</ref>
*The table below differentiates between the three main groups of meningioma according to WHO histological classification:<ref name="C">Meningioma. Canadian Cancer Society http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/brain-and-spinal-tumours/meningioma/?region=mb September, 25 2015</ref><ref name="radio">Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25 2015</ref><ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25 2015</ref>


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*Shown below is a series of microscopic images of different subtypes of meningioma:<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25th 2015</ref>
*Shown below is a series of microscopic images of different subtypes of meningioma:<ref name="liber">Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25 2015</ref>


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]

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.[1][2][3] The majority of meningiomas are benign. Development of meningioma is the result of multiple genetic mutations The majority of meningioma cases involve a deletion of the neurofibromatosis 2 gene located on chromosome 22. On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma. On microscopic histopathological analysis, whorled appearance, calcification, and psammoma bodies are characteristic findings of meningioma.[4]

Pathogenesis

  • 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.[1]
  • The majority of meningiomas are benign; however, a minority of cases may develop a malignant nature.
  • The exact pathophysiology of meningioma depends on the histological subtype of the tumor.
  • Meningioma may be located either intradural or extradural along the central nervous system.[5]
  • Meningiomas are frequently found attached to the dura surrounding the brain tissue in several locations that include:[1]

Genetics

  • Development of meningioma is the result of multiple genetic mutations.[1]
  • The majority of meningioma cases involve a deletion of the neurofibromatosis 2 gene located on chromosome 22.
  • Other genes involved in the pathogenesis of meningioma include:

Gross Pathology

  • On gross pathology, a gray, well-circumscribed, dome-shaped mass is a characteristic finding of meningioma.[1]


Gross pathology: Cut surface of a resected meningioma[6]

Microscopic Pathology

  • On microscopic histopathological analysis, whorled appearance, calcification, and psammoma bodies are characteristic findings of of meningioma.[4]
  • The table below differentiates between the three main groups of meningioma according to WHO histological classification:[2][3][4]
Grade Histologic features Image

Benign (Grade I) meningioma

Less then 4 mitosis/10 HPF, no atypia

Grade 1 Meningioma

Atypical (Grade II) meningioma

Brain invasion, 4 or more mitosis/10 HPF, necrosis, increased cell count, high nucleus:cytoplasm ratio, increased nucleoli size, presence of sheeting

Grade 2 menigioma

Anaplastic (Grade III) meningioma

20 or more mitoses/10 HPF, histology may be similar to carcinoma or sarcoma

Grade 3 menigioma


  • Shown below is a series of microscopic images of different subtypes of meningioma:[4]
  • Meningothelial meningioma with syncytial appearance and whorl formation

    Meningothelial meningioma with syncytial appearance and whorl formation

  • Meningothelial meningioma with psammoma body

    Meningothelial meningioma with psammoma body

  • Meningothelial meningioma with onion bulb formation

    Meningothelial meningioma with onion bulb formation

  • Meningothelial meningioma on HPS stain

    Meningothelial meningioma on HPS stain

  • Fibrous meningioma with spindle cells in parallel bundles

    Fibrous meningioma with spindle cells in parallel bundles

  • Fibrous meingioma on EMA stain

    Fibrous meingioma on EMA stain

  • Transitional meningioma with coexisting lobular and fasicular growth patterns

    Transitional meningioma with coexisting lobular and fasicular growth patterns

  • Psammomatous meningioma with numerous psammoma bodies

    Psammomatous meningioma with numerous psammoma bodies

  • Angiomatous meningioma with hyalinized vessels

    Angiomatous meningioma with hyalinized vessels

  • Microcystic meningioma with cystic appearance and increased pleomorphism of the elongated cells

    Microcystic meningioma with cystic appearance and increased pleomorphism of the elongated cells

  • Secretory meningioma with secretory granules

    Secretory meningioma with secretory granules

  • Secretory meningioma with PAS stain positive secretory granules

    Secretory meningioma with PAS stain positive secretory granules

  • Chordoid meningioma with myxoid appearance

    Chordoid meningioma with myxoid appearance

  • Papillary meningioma with discohesive tumor cells located around a fibrovascular core and perivascular pseudo- rosettes

    Papillary meningioma with discohesive tumor cells located around a fibrovascular core and perivascular pseudo- rosettes

  • Rhabdoid meningioma with abundant cytoplasm and cross-striations

    Rhabdoid meningioma with abundant cytoplasm and cross-striations

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 Meningioma. Wikipedia(2015) https://en.wikipedia.org/wiki/Meningioma#cite_note-pmid7731706-9 Accessed on September, 25 2015
  2. ↑ 2.0 2.1 Meningioma. Canadian Cancer Society http://www.cancer.ca/en/cancer-information/cancer-type/brain-spinal/brain-and-spinal-tumours/meningioma/?region=mb September, 25 2015
  3. ↑ 3.0 3.1 3.2 Meningeoma. Radiopaedia(2015)http://radiopaedia.org/articles/meningioma Accessed on September, 25 2015
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview accessed on September, 25 2015
  5. ↑ Meningioma. Libre Pathology(2015) http://librepathology.org/wiki/index.php/Meningioma#Quick_overview Accessed on September, 25 2015
  6. ↑ Image courtesy of Dr Dharam Ramnani Radiopaedia(original file "here"). Creative Commons BY-SA-NC


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