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[[Category:Diagnosis]]
[[Category:Breast pathology]]
e421ea212eec11d2726a010e4baa89cc84a9ecf74c12c5cc06435e8bc4e28b7e librepath-dcis.txt
{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Ependymoma_H%26E.jpg
| Width =
| Caption = Ependymoma grade II WHO. [[H&E stain]]
| Synonyms =
| Micro = Perivascular pseudorosettes, ependymal rosettes
| Subtypes = Tanycytic, Clear cell, Papillary, Cellular
| LMDDx = [[Subependymoma]], [[Glioblastoma]], [[Pilocytic astrocytoma]], [[Oligodendroglioma]]
| Stains =
| IHC = GFAP +ve
| EM =
| Molecular =
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| Grossing =
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| Assdx =
| Syndromes =
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| Prognosis = intermediate to poor (WHO Grades II & III)
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}}
'''Ependymoma''' is a [[neuropathology tumour]].
==General==
*Called the forgotten glial tumour.
*Anatomic location and molecular data is essential for tumor diagnosis.
Epidemiology:<ref name=Ref_PBoD8_1334>{{Ref PBoD8|1334}}</ref>
*Usual site:
**Adults: usually spinal cord.
**Children: usually posterior fossa.
*May be associated with [[neurofibromatosis type 2]].
There are currently ten main ependymal tumors:<ref name=Ref_WHOCNS_74>{{Ref WHOCNS|74}}</ref>
#Supratentorial [[Subependymoma]]
#Supratentorial ependymoma, ZFTA-fusion positive
#Supratentorial ependymoma, YAP1-fusion positive
#Posterior fossa [[Subependymoma]]
#Posterior fossa ependymoma group A
#Posterior fossa ependymoma group B
#Spinal [[Subependymoma]]
#Spinal ependymoma
#Spinal ependymoma, MYCN-amplified
#[[Myxopapillary ependymoma]]
Ependymoma, NOS (not otherwise specified): Molecular analysis still missing.
Ependymoma, NEC (not elsewhere classfied): Tumor cannot assigned to any of the defined entities.
Note: Molecularly defined ependymomas can be still graded as CNS grade 2 or 3 depending on histological features.
*Depreceated terminologies:
**Papillary ependymoma.
**Clear cell ependymoma.
**Tanycytic ependymoma.
**Cellular ependymoma.
**Ependymoma, RELA fusion-positive.<ref>{{Cite journal | last1 = Parker | first1 = M. | last2 = Mohankumar | first2 = KM. | last3 = Punchihewa | first3 = C. | last4 = Weinlich | first4 = R. | last5 = Dalton | first5 = JD. | last6 = Li | first6 = Y. | last7 = Lee | first7 = R. | last8 = Tatevossian | first8 = RG. | las...
**Anaplastic ependymoma. This is now called CNS grade 3 ependymoma.
==Gross==
*Usually discrete and enhancing.
*Ventricular location, but also within the spinal cord.
*Dissemination possible.
*[[Myxopapillary ependymoma]] classically at [[filum terminale]].
*[[Subependymoma]] typically seen in IVth ventricle.
<gallery>
File:AFIP405711R-EPENDYMOMA.jpg | Radiology (AFIP)
File:AFIP405713G-EPENDYMOMA.jpg | Ependymoma in the fourth ventricle (AFIP)
File:Ependymoma in the fourth ventricle.jpg | Gross (AFIP)
</gallery>
==Microscopic==
==="Classic" ependymoma===
*Come in two CNS WHO grades: 2 and 3.
*Usu. sharply demarcated from surrounding brain parenchyma.
Features:
*Cells have a "tadpole-like" morphology.
**May also be described as ''ice cream cone-shaped''.<ref>[http://www.pathology.vcu.edu/WirSelfInst/tumor-2.html http://www.pathology.vcu.edu/WirSelfInst/tumor-2.html]</ref>
*'''Rosettes''' = circular nuclear free zones/cells arranged in a pseudoglandular fashion; comes in two flavours in ependymoma:
**''Perivascular pseudorosettes'' = (tumour) cells arranged around a blood vessel; nuclei of cells distant from the blood vessel, i.e. rim of cytoplasm (from tumour cells) surround blood vessel (nucleus-free zone); more common than ependymal rosette... but less specific.
**''Ependymal rosette'' ([[AKA]] ''true ependymal rosette'') = rosette has an empty space at the centre - '''key feature'''.
*Nuclear features monotonous, i.e. "boring".<ref>MUN. 6 Oct 2009.</ref>
**There is little variation in size, shape and staining.