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+molecular update
Jensflorian (talk | contribs) (minor update. I suggest moving myxopapillary ependymoma into a separate page) |
Jensflorian (talk | contribs) (+molecular update) |
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#*Typically seen in IVth ventricle | #*Typically seen in IVth ventricle | ||
==Gross== | |||
*Usually discrete and enhancing. | |||
*Ventricular location, but also within the spinal cord. | |||
*Dissemination possible. | |||
<gallery> | |||
File:AFIP405711R-EPENDYMOMA.jpg | Radiology (AFIP) | |||
File:Ependymoma in the fourth ventricle.jpg | Gross (AFIP) | |||
</gallery> | |||
==Microscopic== | ==Microscopic== | ||
| Line 82: | Line 89: | ||
==IHC== | ==IHC== | ||
*Reticulin. | *Reticulin. | ||
*GFAP. | *GFAP+ve. | ||
*MIB1. | *MIB1. | ||
*EMA (dots and rings). | |||
==Molecular== | |||
Two distinct molecular subgroups exist in the posterior fossa:<ref>{{Cite journal | last1 = Witt | first1 = H. | last2 = Mack | first2 = SC. | last3 = Ryzhova | first3 = M. | last4 = Bender | first4 = S. | last5 = Sill | first5 = M. | last6 = Isserlin | first6 = R. | last7 = Benner | first7 = A. | last8 = Hielscher | first8 = T. | last9 = Milde | first9 = T. | title = Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma. | journal = Cancer Cell | volume = 20 | issue = 2 | pages = 143-57 | month = Aug | year = 2011 | doi = 10.1016/j.ccr.2011.07.007 | PMID = 21840481 }}</ref> | |||
* Group A ependymomas: | |||
**typically found in children. | |||
**laterally. | |||
**relatively unfavorable clinical outcome. | |||
**CpG island methylator phenotype.<ref>{{Cite journal | last1 = Mack | first1 = SC. | last2 = Witt | first2 = H. | last3 = Piro | first3 = RM. | last4 = Gu | first4 = L. | last5 = Zuyderduyn | first5 = S. | last6 = Stütz | first6 = AM. | last7 = Wang | first7 = X. | last8 = Gallo | first8 = M. | last9 = Garzia | first9 = L. | title = Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. | journal = Nature | volume = 506 | issue = 7489 | pages = 445-50 | month = Feb | year = 2014 | doi = 10.1038/nature13108 | PMID = 24553142 }}</ref> | |||
* Group B ependymomas: | |||
**typically adults. | |||
**midline. | |||
**relatively favorable clinical outcomes. | |||
**gene expression profiles similar to that of spinal cord ependymomas. | |||
**increased Chromosomal 1q gains. <ref>{{Cite journal | last1 = Korshunov | first1 = A. | last2 = Witt | first2 = H. | last3 = Hielscher | first3 = T. | last4 = Benner | first4 = A. | last5 = Remke | first5 = M. | last6 = Ryzhova | first6 = M. | last7 = Milde | first7 = T. | last8 = Bender | first8 = S. | last9 = Wittmann | first9 = A. | title = Molecular staging of intracranial ependymoma in children and adults. | journal = J Clin Oncol | volume = 28 | issue = 19 | pages = 3182-90 | month = Jul | year = 2010 | doi = 10.1200/JCO.2009.27.3359 | PMID = 20516456 }}</ref> | |||
Supratentorial ependymomas have also a distinct profile: | |||
*70 % of these ependymomas have recurrent gene fusions involving RELA and C11orf95<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. | last9 = Phoenix | first9 = TN. | title = C11orf95-RELA fusions drive oncogenic NF-κB signalling in ependymoma. | journal = Nature | volume = 506 | issue = 7489 | pages = 451-5 | month = Feb | year = 2014 | doi = 10.1038/nature13109 | PMID = 24553141 }}</ref> | |||
*EPHB2 amplifications and CDKN2A deletions in a subset of these tumors<ref>{{Cite journal | last1 = Philip-Hollingsworth | first1 = S. | last2 = Hollingsworth | first2 = RI. | last3 = Dazzo | first3 = FB. | title = Host-range related structural features of the acidic extracellular polysaccharides of Rhizobium trifolii and Rhizobium leguminosarum. | journal = J Biol Chem | volume = 264 | issue = 3 | pages = 1461-6 | month = Jan | year = 1989 | doi = | PMID = 2912966 }}</ref> | |||
Note: Molecular subgroups have no treatment implications (at the moment). | |||
==See also== | ==See also== | ||