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<meta name="DC.Title" content="Ependymal Tumors: Overview of the Recent World Health Organization Histopathologic and Genetic Updates with an Imaging Characteristic" />
<meta name="DC.Identifier" content="10.3174/ajnr.A8237" />
<meta name="DC.Date" content="2024-11-01" />
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<meta name="DC.Description" content="The 2021 World Health Organization Classification of Tumors of the Central Nervous System (CNS5), introduced significant changes, impacting tumors ranging from glial to ependymal neoplasms. Ependymal tumors were previously classified and graded based on histopathology, which had limited clinical and prognostic utility. The updated CNS5 classification now divides ependymomas into 10 subgroups based on anatomic location (supratentorial, posterior fossa, and spinal compartment) and genomic markers. Supratentorial tumors are defined by zinc finger translocation associated ( ZFTA ) (formerly v-rel avian reticuloendotheliosis viral oncogene [ RELA ]), or yes-associated protein 1 ( YAP1 ) fusion; posterior fossa tumors are classified into groups A (PFA) and B (PFB), spinal ependymomas are defined by MYCN amplification. Subependymomas are present across all these anatomic compartments. The new classification kept an open category of “not elsewhere classified” or “not otherwise specified” if no pathogenic gene fusion is identified or if the molecular diagnosis is not feasible. Although there is significant overlap in the imaging findings of these tumors, a neuroradiologist needs to be familiar with updated CNS5 classification to understand tumor behavior, for example, the higher tendency for tumor recurrence along the dural flap for ZFTA fusion-positive ependymomas. On imaging, supratentorial ZFTA -fused ependymomas are preferentially located in the cerebral cortex, carrying predominant cystic components. YAP1-MAMLD1 -fused ependymomas are intra- or periventricular with prominent multinodular solid components and have significantly better prognosis than ZFTA -fused counterparts. PFA ependymomas are aggressive paramedian masses with frequent calcification, seen in young children, originating from the lateral part of the fourth ventricular roof. PFB ependymomas are usually midline, noncalcified solid-cystic masses seen in adolescents and young adults arising from the fourth ventricular floor. PFA has a poorer prognosis, higher recurrence, and higher metastatic rate than PFB. Myxopapillary spinal ependymomas are now considered grade II due to high recurrence rates. Spinal- MYCN ependymomas are aggressive tumors with frequent leptomeningeal spread, relapse, and poor prognosis. Subependymomas are noninvasive, intraventricular, slow-growing benign tumors with an excellent prognosis. Currently, the molecular classification does not enhance the clinicopathologic understanding of subependymoma and myxopapillary categories. However, given the molecular advancements, this will likely change in the future. This review provides an updated molecular classification of ependymoma, discusses the individual imaging characteristics, and briefly outlines the latest targeted molecular therapies.

CNS5
:   2021 World Health Organization Classification of Tumors of the Central Nervous System
EPN
:   ependymomas
EZHIP
:   enhancer of zeste homolog inhibitory protein
MAMLD1 
:   mastermind-like domain containing 1 gene
MPE
:   myxopapillary ependymoma
NEC
:   not elsewhere classified
NF2
:   neurofibromatosis type 2
NOS
:   not otherwise specified
OS
:   overall survival
PARP
:   poly adenosine diphosphate ribose polymerase
PFA
:   posterior fossa A
PFB
:   posterior fossa B
PFS
:   progression-free survival
RELA 
:   v-rel avian reticuloendotheliosis viral oncogene
ST
:   supratentorial
WHO
:   World Health Organization
YAP1 
:   yes-associated protein 1
ZFTA 
:   zinc finger translocation associated" />
<meta name="DC.Contributor" content="Neetu Soni" />
<meta name="DC.Contributor" content="Manish Ora" />
<meta name="DC.Contributor" content="Girish Bathla" />
<meta name="DC.Contributor" content="Amit Desai" />
<meta name="DC.Contributor" content="Vivek Gupta" />
<meta name="DC.Contributor" content="Amit Agarwal" />
<meta name="article:published_time" content="2024-11-01" />
<meta name="article:section" content="Brain Tumor Imaging" />
<meta name="citation_title" content="Ependymal Tumors: Overview of the Recent World Health Organization Histopathologic and Genetic Updates with an Imaging Characteristic" />
<meta name="citation_abstract" lang="en" content="&lt;h3&gt;SUMMARY:&lt;/h3&gt;
&lt;p&gt;The 2021 World Health Organization Classification of Tumors of the Central Nervous System (CNS5), introduced significant changes, impacting tumors ranging from glial to ependymal neoplasms. Ependymal tumors were previously classified and graded based on histopathology, which had limited clinical and prognostic utility. The updated CNS5 classification now divides ependymomas into 10 subgroups based on anatomic location (supratentorial, posterior fossa, and spinal compartment) and genomic markers. Supratentorial tumors are defined by zinc finger translocation associated (&lt;i&gt;ZFTA&lt;/i&gt;) (formerly v-rel avian reticuloendotheliosis viral oncogene [&lt;i&gt;RELA&lt;/i&gt;]), or yes-associated protein 1 (&lt;i&gt;YAP1&lt;/i&gt;) fusion; posterior fossa tumors are classified into groups A (PFA) and B (PFB), spinal ependymomas are defined by &lt;i&gt;MYCN&lt;/i&gt; amplification. Subependymomas are present across all these anatomic compartments. The new classification kept an open category of “not elsewhere classified” or “not otherwise specified” if no pathogenic gene fusion is identified or if the molecular diagnosis is not feasible. Although there is significant overlap in the imaging findings of these tumors, a neuroradiologist needs to be familiar with updated CNS5 classification to understand tumor behavior, for example, the higher tendency for tumor recurrence along the dural flap for &lt;i&gt;ZFTA&lt;/i&gt; fusion-positive ependymomas. On imaging, supratentorial &lt;i&gt;ZFTA&lt;/i&gt;-fused ependymomas are preferentially located in the cerebral cortex, carrying predominant cystic components. &lt;i&gt;YAP1-MAMLD1&lt;/i&gt;-fused ependymomas are intra- or periventricular with prominent multinodular solid components and have significantly better prognosis than &lt;i&gt;ZFTA&lt;/i&gt;-fused counterparts. PFA ependymomas are aggressive paramedian masses with frequent calcification, seen in young children, originating from the lateral part of the fourth ventricular roof. PFB ependymomas are usually midline, noncalcified solid-cystic masses seen in adolescents and young adults arising from the fourth ventricular floor. PFA has a poorer prognosis, higher recurrence, and higher metastatic rate than PFB. Myxopapillary spinal ependymomas are now considered grade II due to high recurrence rates. Spinal-&lt;i&gt;MYCN&lt;/i&gt; ependymomas are aggressive tumors with frequent leptomeningeal spread, relapse, and poor prognosis. Subependymomas are noninvasive, intraventricular, slow-growing benign tumors with an excellent prognosis. Currently, the molecular classification does not enhance the clinicopathologic understanding of subependymoma and myxopapillary categories. However, given the molecular advancements, this will likely change in the future. This review provides an updated molecular classification of ependymoma, discusses the individual imaging characteristics, and briefly outlines the latest targeted molecular therapies.&lt;/p&gt;" />
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<meta name="twitter:title" content="Ependymal Tumors: Overview of the Recent World Health Organization Histopathologic and Genetic Updates with an Imaging Characteristic" />
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<meta name="twitter:description" content="The 2021 World Health Organization Classification of Tumors of the Central Nervous System (CNS5), introduced significant changes, impacting tumors ranging from glial to ependymal neoplasms. Ependymal tumors were previously classified and graded based on histopathology, which had limited clinical and prognostic utility. The updated CNS5 classification now divides ependymomas into 10 subgroups based on anatomic location (supratentorial, posterior fossa, and spinal compartment) and genomic markers. Supratentorial tumors are defined by zinc finger translocation associated ( ZFTA ) (formerly v-rel avian reticuloendotheliosis viral oncogene [ RELA ]), or yes-associated protein 1 ( YAP1 ) fusion; posterior fossa tumors are classified into groups A (PFA) and B (PFB), spinal ependymomas are defined by MYCN amplification. Subependymomas are present across all these anatomic compartments. The new classification kept an open category of “not elsewhere classified” or “not otherwise specified” if no pathogenic gene fusion is identified or if the molecular diagnosis is not feasible. Although there is significant overlap in the imaging findings of these tumors, a neuroradiologist needs to be familiar with updated CNS5 classification to understand tumor behavior, for example, the higher tendency for tumor recurrence along the dural flap for ZFTA fusion-positive ependymomas. On imaging, supratentorial ZFTA -fused ependymomas are preferentially located in the cerebral cortex, carrying predominant cystic components. YAP1-MAMLD1 -fused ependymomas are intra- or periventricular with prominent multinodular solid components and have significantly better prognosis than ZFTA -fused counterparts. PFA ependymomas are aggressive paramedian masses with frequent calcification, seen in young children, originating from the lateral part of the fourth ventricular roof. PFB ependymomas are usually midline, noncalcified solid-cystic masses seen in adolescents and young adults arising from the fourth ventricular floor. PFA has a poorer prognosis, higher recurrence, and higher metastatic rate than PFB. Myxopapillary spinal ependymomas are now considered grade II due to high recurrence rates. Spinal- MYCN ependymomas are aggressive tumors with frequent leptomeningeal spread, relapse, and poor prognosis. Subependymomas are noninvasive, intraventricular, slow-growing benign tumors with an excellent prognosis. Currently, the molecular classification does not enhance the clinicopathologic understanding of subependymoma and myxopapillary categories. However, given the molecular advancements, this will likely change in the future. This review provides an updated molecular classification of ependymoma, discusses the individual imaging characteristics, and briefly outlines the latest targeted molecular therapies.

CNS5
:   2021 World Health Organization Classification of Tumors of the Central Nervous System
EPN
:   ependymomas
EZHIP
:   enhancer of zeste homolog inhibitory protein
MAMLD1 
:   mastermind-like domain containing 1 gene
MPE
:   myxopapillary ependymoma
NEC
:   not elsewhere classified
NF2
:   neurofibromatosis type 2
NOS
:   not otherwise specified
OS
:   overall survival
PARP
:   poly adenosine diphosphate ribose polymerase
PFA
:   posterior fossa A
PFB
:   posterior fossa B
PFS
:   progression-free survival
RELA 
:   v-rel avian reticuloendotheliosis viral oncogene
ST
:   supratentorial
WHO
:   World Health Organization
YAP1 
:   yes-associated protein 1
ZFTA 
:   zinc finger translocation associated" />
<meta name="og-title" property="og:title" content="Ependymal Tumors: Overview of the Recent World Health Organization Histopathologic and Genetic Updates with an Imaging Characteristic" />
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<meta name="og-description" property="og:description" content="The 2021 World Health Organization Classification of Tumors of the Central Nervous System (CNS5), introduced significant changes, impacting tumors ranging from glial to ependymal neoplasms. Ependymal tumors were previously classified and graded based on histopathology, which had limited clinical and prognostic utility. The updated CNS5 classification now divides ependymomas into 10 subgroups based on anatomic location (supratentorial, posterior fossa, and spinal compartment) and genomic markers. Supratentorial tumors are defined by zinc finger translocation associated ( ZFTA ) (formerly v-rel avian reticuloendotheliosis viral oncogene [ RELA ]), or yes-associated protein 1 ( YAP1 ) fusion; posterior fossa tumors are classified into groups A (PFA) and B (PFB), spinal ependymomas are defined by MYCN amplification. Subependymomas are present across all these anatomic compartments. The new classification kept an open category of “not elsewhere classified” or “not otherwise specified” if no pathogenic gene fusion is identified or if the molecular diagnosis is not feasible. Although there is significant overlap in the imaging findings of these tumors, a neuroradiologist needs to be familiar with updated CNS5 classification to understand tumor behavior, for example, the higher tendency for tumor recurrence along the dural flap for ZFTA fusion-positive ependymomas. On imaging, supratentorial ZFTA -fused ependymomas are preferentially located in the cerebral cortex, carrying predominant cystic components. YAP1-MAMLD1 -fused ependymomas are intra- or periventricular with prominent multinodular solid components and have significantly better prognosis than ZFTA -fused counterparts. PFA ependymomas are aggressive paramedian masses with frequent calcification, seen in young children, originating from the lateral part of the fourth ventricular roof. PFB ependymomas are usually midline, noncalcified solid-cystic masses seen in adolescents and young adults arising from the fourth ventricular floor. PFA has a poorer prognosis, higher recurrence, and higher metastatic rate than PFB. Myxopapillary spinal ependymomas are now considered grade II due to high recurrence rates. Spinal- MYCN ependymomas are aggressive tumors with frequent leptomeningeal spread, relapse, and poor prognosis. Subependymomas are noninvasive, intraventricular, slow-growing benign tumors with an excellent prognosis. Currently, the molecular classification does not enhance the clinicopathologic understanding of subependymoma and myxopapillary categories. However, given the molecular advancements, this will likely change in the future. This review provides an updated molecular classification of ependymoma, discusses the individual imaging characteristics, and briefly outlines the latest targeted molecular therapies.

CNS5
:   2021 World Health Organization Classification of Tumors of the Central Nervous System
EPN
:   ependymomas
EZHIP
:   enhancer of zeste homolog inhibitory protein
MAMLD1 
:   mastermind-like domain containing 1 gene
MPE
:   myxopapillary ependymoma
NEC
:   not elsewhere classified
NF2
:   neurofibromatosis type 2
NOS
:   not otherwise specified
OS
:   overall survival
PARP
:   poly adenosine diphosphate ribose polymerase
PFA
:   posterior fossa A
PFB
:   posterior fossa B
PFS
:   progression-free survival
RELA 
:   v-rel avian reticuloendotheliosis viral oncogene
ST
:   supratentorial
WHO
:   World Health Organization
YAP1 
:   yes-associated protein 1
ZFTA 
:   zinc finger translocation associated" />
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    	<div class="highwire-cite-metadata"><span  class="highwire-cite-metadata-journal highwire-cite-metadata">American Journal of Neuroradiology </span><span  class="highwire-cite-metadata-date highwire-cite-metadata">Nov 2024, </span><span  class="highwire-cite-metadata-volume highwire-cite-metadata">45 </span><span  class="highwire-cite-metadata-issue highwire-cite-metadata">(11) </span><span  class="highwire-cite-metadata-pages highwire-cite-metadata">1624-1634; </span><span  class="highwire-cite-metadata-doi highwire-cite-metadata"><span class="label">DOI:</span> 10.3174/ajnr.A8237 </span></div>
  
  
  
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