Difference between revisions of "Ependymoma"
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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== | ||
Revision as of 09:01, 16 April 2015
Ependymoma is a neuropathology tumour.
General
- Called the forgotten glial tumour.
Epidemiology:[1]
- Usual site:
- Adults: usu. spinal cord.
- Children: usu. posterior fossa.
- May be assoc. with neurofibromatosis 2.
There are four main ependymal tumors:
- Ependymoma (not otherwise specified).
- Other flavours:[2]
- Cellular ependymoma.
- Papillary ependymoma.
- Clear cell ependymoma.
- Tanycytic ependymoma.
- Other flavours:[2]
- Anaplastic ependymoma.
- Myxopapillary ependymoma.
- Classically at filum terminale.
- Subependymoma
- Typically seen in IVth ventricle
Gross
- Usually discrete and enhancing.
- Ventricular location, but also within the spinal cord.
- Dissemination possible.
Radiology (AFIP)
Gross (AFIP)
Microscopic
Classic ependymoma
Features:
- Cells have a "tadpole-like" morphology.
- May also be described as ice cream cone-shaped.[3]
- 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".[4]
- There is little variation in size, shape and staining.
DDx (classic ependymoma):
- Subependymoma.
- Glioblastoma (GBM).
- Invasive border = GBM; circumscribed border of lesion = ependymoma.
Images
www:
- Ependymoma (flickr.com).
- Ependymoma - ependymal rosettes (ajnr.org).
- Anaplastic ependymoma - case 1 (upmc.edu).
- Anaplastic ependymoma - case 2 (upmc.edu).
Ependymoma - intermed. mag. (WC)
Ependymoma - low mag. (WC)
Myxopapillary ependymoma
Features:
- Perivascular pseudorosettes:
- Myxoid material surround blood vessels.
- Myxoid material surrounded by tumour cells.
- Myxoid material surround blood vessels.
Images:
- Myxopapillary ependymoma - high mag. (WC).
- Myxopapillary ependymoma (bmj.com) - part of careers.bmj.com article on paediatric pathology.
- Myxopapillary ependymoma - cytology (WC).
- Myxopapillary ependymoma - several images (upmc.edu).
Grading
Easy:
- Subependymoma = WHO grade I.
- Myxopapillary ependymoma = WHO grade I.
Not-so-easy:
- Classic ependymoma = WHO grade II.
- Anaplastic ependymoma = WHO grade III.
Grade II vs. Grade III:
- Cellular density.
- Mitoses.
- Necrosis.
- Microvascular proliferation.
Notes:
- Many tumours fall between grade II and grade III. These are called "indeterminate" by many.
IHC
- Reticulin.
- GFAP+ve.
- MIB1.
- EMA (dots and rings).
Molecular
Two distinct molecular subgroups exist in the posterior fossa:[5]
- Group A ependymomas:
- typically found in children.
- laterally.
- relatively unfavorable clinical outcome.
- CpG island methylator phenotype.[6]
- Group B ependymomas:
- typically adults.
- midline.
- relatively favorable clinical outcomes.
- gene expression profiles similar to that of spinal cord ependymomas.
- increased Chromosomal 1q gains. [7]
Supratentorial ependymomas have also a distinct profile:
- 70 % of these ependymomas have recurrent gene fusions involving RELA and C11orf95[8]
- EPHB2 amplifications and CDKN2A deletions in a subset of these tumors[9]
Note: Molecular subgroups have no treatment implications (at the moment).
See also
References
- ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1334. ISBN 978-1416031215.
- ↑ URL: http://emedicine.medscape.com/article/1744030-overview. Accessed on: 17 January 2012.
- ↑ http://www.pathology.vcu.edu/WirSelfInst/tumor-2.html
- ↑ MUN. 6 Oct 2009.
- ↑ Witt, H.; Mack, SC.; Ryzhova, M.; Bender, S.; Sill, M.; Isserlin, R.; Benner, A.; Hielscher, T. et al. (Aug 2011). "Delineation of two clinically and molecularly distinct subgroups of posterior fossa ependymoma.". Cancer Cell 20 (2): 143-57. doi:10.1016/j.ccr.2011.07.007. PMID 21840481.
- ↑ Mack, SC.; Witt, H.; Piro, RM.; Gu, L.; Zuyderduyn, S.; Stütz, AM.; Wang, X.; Gallo, M. et al. (Feb 2014). "Epigenomic alterations define lethal CIMP-positive ependymomas of infancy.". Nature 506 (7489): 445-50. doi:10.1038/nature13108. PMID 24553142.
- ↑ Korshunov, A.; Witt, H.; Hielscher, T.; Benner, A.; Remke, M.; Ryzhova, M.; Milde, T.; Bender, S. et al. (Jul 2010). "Molecular staging of intracranial ependymoma in children and adults.". J Clin Oncol 28 (19): 3182-90. doi:10.1200/JCO.2009.27.3359. PMID 20516456.
- ↑ Parker, M.; Mohankumar, KM.; Punchihewa, C.; Weinlich, R.; Dalton, JD.; Li, Y.; Lee, R.; Tatevossian, RG. et al. (Feb 2014). "C11orf95-RELA fusions drive oncogenic NF-κB signalling in ependymoma.". Nature 506 (7489): 451-5. doi:10.1038/nature13109. PMID 24553141.
- ↑ Philip-Hollingsworth, S.; Hollingsworth, RI.; Dazzo, FB. (Jan 1989). "Host-range related structural features of the acidic extracellular polysaccharides of Rhizobium trifolii and Rhizobium leguminosarum.". J Biol Chem 264 (3): 1461-6. PMID 2912966.