Ependymoma: Difference between revisions

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== Definition ==
== Definition ==
Ependymomas are uncommon tumours of the central nervous system which are therefore found in the brain or spinal cord. Glial cells are most commonly effected. Like most other types of brain tumour, the World Health Organization (WHO) classifies the subtypes of ependymal tumour (listed below) using a numerical grading scale. In general terms, a lower grade of one usually means that the tumour cell grows relatively slowly, is less likely to spread and looks substantially similar to a healthy cell under the microscope. On the other hand, the higher the grading of a tumour cell (two-three-four), the less it shares with healthy cells in terms of appearance, the faster it grows, and the more likely it is to spread to other parts of the brain or spinal cord. Listed below are the different subtypes of ependymoma along with their WHO grade:
Ependymomas are tumors of the central nervous system (CNS), which means that they originate in either the brain or spine. Tumors of glial cells are the most common and are known as gliomas. They effect both adults and children but are most common in children, Tumors are classified by the World Health Organization (WHO) Classification of Tumors of the Nervous System as grades I, II, and III based on their grade of anaplasia (cell disorganisation)<ref name=":0">Cern-foundation.org. (2017). ''Ependymoma Basics | CERN Foundation''. [online] Available at: <nowiki>http://www.cern-foundation.org/education/ependymoma-basics</nowiki> [Accessed 24 Aug. 2017].
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== Clinically Relevant Anatomy<br>  ==
== Clinically Relevant Anatomy ==


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=== Glial cells ===
Glial cells are a cell type that are found between nerve cells that share a general function of holding the CNS together. There are more glial cells than nerve cells and thereby constitute half of the total volume of the CNS. The four types of glial cells are: oligodenrocytes, microglia, epndymal cells and astrocytes.<ref>Palastanga, N., Field, D. and Soames, R. (2012). ''Anatomy and Human Movement: Structure and Function''. 6th ed. Burlington: Elsevier Science.</ref>


== Pathological Process  ==
=== Ventricals ===
Ventricals and the central canal of the spinal cord are fluid-filled spaces within the CNS that contain cerebrospinal fluid. Both are common locations for tumours.<ref name=":0" />


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=== Tentorium Cerebelli ===
Tentorium Cerebelli is an extension of the dura matter that separates the cerebellum from the inferior aspect of the occipital lobes. Tumour below the tentorium are called infratentorial and those above are called supratentorial.<ref name=":0" />
 
== Pathological Process ==


== Clinical Presentation  ==
== Clinical Presentation  ==
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== Diagnostic Procedures  ==
== Diagnostic Procedures  ==


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Tumors are classified and graded according to their appearance when viewed through the microscope with the most severe tumour associated with a higher grade.
 
=== World Health Organization (WHO) Classification of Tumors of the Nervous System ===
 
==== Myxopapillary ependymoma (WHO grade I) ====
This entity is characterised by cuboidal tumour cells, with GFAP expression and lack of cytokeratin expression, surrounding blood vessels in a mucoid matrix. Mitotic activity is very low or absent. 2.1.2.


== Outcome Measures  ==
==== Subependymoma (WHO grade I) ====
Subependymoma has isomorphic nuclei in an abundant and dense fibrillary matrix with frequent microcysts; mitoses are very rare or absent. 2.1.3.


add links to outcome measures here (see [[Outcome Measures|Outcome Measures Database]])
==== Ependymoma (WHO grade II) ====
This neoplasm has moderate cellularity; mitoses are rare or absent and nuclear morphology is monomorphic. Key histological features are perivascular pseudorosettes and ependymal rosettes[14]. Four histological variants have been described: cellular ependymoma, which has hypercellularity and increased mitotic rate, papillary ependymoma, clear cell ependymoma and tanycytic ependymoma.


== Management / Interventions<br> ==
==== Anaplastic ependymoma (WHO grade III) ====
This tumour is characterised by hypercellularity, cellular and nuclear pleomorphism, frequent mitosis, pseudopalisading necrosis and endothelial proliferation. The latter two criteria do not appear to be independently related to prognosis [15]. Perivascular rosettes are a histological hallmark.<ref>Reni, M., Gatta, G., Mazza, E. and Vecht, C. (2007). Ependymoma. ''Critical Reviews in Oncology/Hematology'', 63(1), pp.81-89.
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== Management ==
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add text here relating to management approaches to the condition<br>  



Revision as of 18:20, 24 August 2017

Original Editor - George Prudden Lead Editors  

Definition[edit | edit source]

Ependymomas are tumors of the central nervous system (CNS), which means that they originate in either the brain or spine. Tumors of glial cells are the most common and are known as gliomas. They effect both adults and children but are most common in children, Tumors are classified by the World Health Organization (WHO) Classification of Tumors of the Nervous System as grades I, II, and III based on their grade of anaplasia (cell disorganisation)[1]

Clinically Relevant Anatomy[edit | edit source]

Glial cells[edit | edit source]

Glial cells are a cell type that are found between nerve cells that share a general function of holding the CNS together. There are more glial cells than nerve cells and thereby constitute half of the total volume of the CNS. The four types of glial cells are: oligodenrocytes, microglia, epndymal cells and astrocytes.[2]

Ventricals[edit | edit source]

Ventricals and the central canal of the spinal cord are fluid-filled spaces within the CNS that contain cerebrospinal fluid. Both are common locations for tumours.[1]

Tentorium Cerebelli[edit | edit source]

Tentorium Cerebelli is an extension of the dura matter that separates the cerebellum from the inferior aspect of the occipital lobes. Tumour below the tentorium are called infratentorial and those above are called supratentorial.[1]

Pathological Process[edit | edit source]

Clinical Presentation[edit | edit source]

add text here relating to the clinical presentation of the condition

Diagnostic Procedures[edit | edit source]

Tumors are classified and graded according to their appearance when viewed through the microscope with the most severe tumour associated with a higher grade.

World Health Organization (WHO) Classification of Tumors of the Nervous System[edit | edit source]

Myxopapillary ependymoma (WHO grade I)[edit | edit source]

This entity is characterised by cuboidal tumour cells, with GFAP expression and lack of cytokeratin expression, surrounding blood vessels in a mucoid matrix. Mitotic activity is very low or absent. 2.1.2.

Subependymoma (WHO grade I)[edit | edit source]

Subependymoma has isomorphic nuclei in an abundant and dense fibrillary matrix with frequent microcysts; mitoses are very rare or absent. 2.1.3.

Ependymoma (WHO grade II)[edit | edit source]

This neoplasm has moderate cellularity; mitoses are rare or absent and nuclear morphology is monomorphic. Key histological features are perivascular pseudorosettes and ependymal rosettes[14]. Four histological variants have been described: cellular ependymoma, which has hypercellularity and increased mitotic rate, papillary ependymoma, clear cell ependymoma and tanycytic ependymoma.

Anaplastic ependymoma (WHO grade III)[edit | edit source]

This tumour is characterised by hypercellularity, cellular and nuclear pleomorphism, frequent mitosis, pseudopalisading necrosis and endothelial proliferation. The latter two criteria do not appear to be independently related to prognosis [15]. Perivascular rosettes are a histological hallmark.[3]

Management[edit | edit source]

add text here relating to management approaches to the condition

Differential Diagnosis
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add text here relating to the differential diagnosis of this condition

Key Evidence[edit | edit source]

add text here relating to key evidence with regards to any of the above headings

Resources
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add appropriate resources here

Case Studies[edit | edit source]

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References[edit | edit source]

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  1. 1.0 1.1 1.2 Cern-foundation.org. (2017). Ependymoma Basics | CERN Foundation. [online] Available at: http://www.cern-foundation.org/education/ependymoma-basics [Accessed 24 Aug. 2017].
  2. Palastanga, N., Field, D. and Soames, R. (2012). Anatomy and Human Movement: Structure and Function. 6th ed. Burlington: Elsevier Science.
  3. Reni, M., Gatta, G., Mazza, E. and Vecht, C. (2007). Ependymoma. Critical Reviews in Oncology/Hematology, 63(1), pp.81-89.