Ependymoma
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- updated 19 Dec 2022:
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Ependymomas represent a relatively broad group of glial tumours most often arising from the lining of the ventricles of the brain or the central canal of the spinal cord. They account for ~5% of all neuroepithelial neoplasms, ~10% of all paediatric brain tumours and up to 33% of brain tumours occurring in those less than 3 years of age.
Ependymal tumours can occur anywhere within the neuraxis, but distribution and molecular characteristics are fairly site-dependent, divided into three groups depending on the anatomical compartment in which they are found 11,13,15:
- posterior fossa (60%)
- supratentorial (30%)
- spinal cord (10%)
In addition to subependymomas, that can be encountered in all three compartments, DNA-methylation profiling divides ependymomas on the basis of their molecular characteristics and their location as differing in epidemiology and prognosis. As such, location forms the basis of the current (2021) WHO classification 11,13:
- posterior fossa
-
posterior fossa ependymoma - NEC or NOS
- posterior fossa group A (PFA) ependymoma
- posterior fossa group B (PFB) ependymoma
-
posterior fossa ependymoma - NEC or NOS
- supratentorial compartment
-
supratentorial ependymoma - NEC or NOS
- supratentorial ependymoma, ZFTA fusion-positive
- supratentorial ependymoma, YAP1 fusion-positive
-
supratentorial ependymoma - NEC or NOS
- spinal cord/canal
-
spinal ependymoma - NEC or NOS
- spinal ependymoma, MYCN-amplified
- myxopapillary ependymoma
-
spinal ependymoma - NEC or NOS
-<p><strong>Ependymomas </strong>represent a relatively broad group of glial tumours most often arising from the lining of the ventricles of the brain or the central canal of the spinal cord. They account for ~5% of all neuroepithelial neoplasms, ~10% of all paediatric brain tumours and up to 33% of brain tumours occurring in those less than 3 years of age. </p><p>Ependymal tumours can occur anywhere within the neuraxis, but distribution and molecular characteristics are fairly site-dependent, divided into three groups depending on the anatomical compartment in which they are found <sup>11,13,15</sup>:</p><ul>-<li>posterior fossa (60%)</li>-<li>supratentorial (30%)</li>-<li>spinal cord (10%)</li>-</ul><p>In addition to <a href="/articles/subependymoma">subependymomas</a>, that can be encountered in all three compartments, <a href="/articles/dna-methylation">DNA-methylation profiling</a> divides ependymomas on the basis of their molecular characteristics and their location as differing in epidemiology and prognosis. As such, location forms the basis of the current (2021) <a href="/articles/who-classification-of-cns-tumours-1">WHO classification</a> <sup>11,13</sup>:</p><ul>-<li>posterior fossa<ul><li>-<a href="/articles/posterior-fossa-ependymoma">posterior fossa ependymoma</a> - <a href="/articles/not-elsewhere-classified-nec">NEC</a> or <a href="/articles/not-otherwise-specified-nos">NOS</a><ul>-<li>posterior fossa group A (PFA) ependymoma</li>-<li>posterior fossa group B (PFB) ependymoma</li>-</ul>-</li></ul>-</li>-<li>supratentorial compartment<ul><li>-<a href="/articles/supratentorial-ependymoma">supratentorial ependymoma</a> - <a href="/articles/not-elsewhere-classified-nec">NEC</a> or <a href="/articles/not-otherwise-specified-nos">NOS</a><ul>-<li>supratentorial ependymoma, ZFTA fusion-positive</li>-<li>supratentorial ependymoma, YAP1 fusion-positive</li>-</ul>-</li></ul>-</li>-<li>spinal cord/canal<ul>-<li>-<a href="/articles/spinal-ependymoma">spinal ependymoma</a> - <a href="/articles/not-elsewhere-classified-nec">NEC</a> or <a href="/articles/not-otherwise-specified-nos">NOS</a><ul><li>spinal ependymoma, MYCN-amplified</li></ul>-</li>-<li><a href="/articles/myxopapillary-ependymoma-1">myxopapillary ependymoma</a></li>-</ul>-</li>- +<p><strong>Ependymomas </strong>represent a relatively broad group of glial tumours most often arising from the lining of the ventricles of the brain or the central canal of the spinal cord. They account for ~5% of all neuroepithelial neoplasms, ~10% of all paediatric brain tumours and up to 33% of brain tumours occurring in those less than 3 years of age. </p><p>Ependymal tumours can occur anywhere within the neuraxis, but distribution and molecular characteristics are fairly site-dependent, divided into three groups depending on the anatomical compartment in which they are found <sup>11,13,15</sup>:</p><ul>
- +<li>posterior fossa (60%)</li>
- +<li>supratentorial (30%)</li>
- +<li>spinal cord (10%)</li>
- +</ul><p>In addition to <a href="/articles/subependymoma">subependymomas</a>, that can be encountered in all three compartments, <a href="/articles/dna-methylation">DNA-methylation profiling</a> divides ependymomas on the basis of their molecular characteristics and their location as differing in epidemiology and prognosis. As such, location forms the basis of the current (2021) <a href="/articles/who-classification-of-cns-tumours-1">WHO classification</a> <sup>11,13</sup>:</p><ul>
- +<li>posterior fossa<ul><li>
- +<a href="/articles/posterior-fossa-ependymoma">posterior fossa ependymoma</a> - <a href="/articles/not-elsewhere-classified-nec">NEC</a> or <a href="/articles/not-otherwise-specified-nos">NOS</a><ul>
- +<li>posterior fossa group A (PFA) ependymoma</li>
- +<li>posterior fossa group B (PFB) ependymoma</li>
- +</ul>
- +</li></ul>
- +</li>
- +<li>supratentorial compartment<ul><li>
- +<a href="/articles/supratentorial-ependymoma">supratentorial ependymoma</a> - <a href="/articles/not-elsewhere-classified-nec">NEC</a> or <a href="/articles/not-otherwise-specified-nos">NOS</a><ul>
- +<li>supratentorial ependymoma, ZFTA fusion-positive</li>
- +<li>supratentorial ependymoma, YAP1 fusion-positive</li>
- +</ul>
- +</li></ul>
- +</li>
- +<li>spinal cord/canal<ul>
- +<li>
- +<a href="/articles/spinal-ependymoma">spinal ependymoma</a> - <a href="/articles/not-elsewhere-classified-nec">NEC</a> or <a href="/articles/not-otherwise-specified-nos">NOS</a><ul><li>spinal ependymoma, MYCN-amplified</li></ul>
- +</li>
- +<li><a href="/articles/myxopapillary-ependymoma-1">myxopapillary ependymoma</a></li>
- +</ul>
- +</li>
Images Changes:
Image 5 MRI (T1 C+) ( create )
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