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*Hyalinized vessels. |
*Calcification. |
*Rare cases with cartilagineous metaplasia.<ref>{{Cite journal | last1 = Wang | first1 = X. | last2 = Zhang | first2 = S. | last3 = Ye | first3 = Y. | last4 = Chen | first4 = Y. | last5 = Liu | first5 = X. | title = Ependymoma with cartilaginous metaplasia might have more aggressive behavior: a case report and literat... |
*Branching capillaries usu. only in supratentorial ependymomas. |
===Supratentorial ependymoma=== |
*Usu. connected to the ventricles. |
*Mostly frontal or temporal lobe. |
*Approx. 1/3 of all ependymal tumours (41% in children). |
*Irregular CM enhancement. |
*YAP1-fused tumors in children oft large at time of diagnosis. |
*Cysts and/or calcification possible. |
*Sharply demarcated from adjacent brain parenchyma. |
*True ependymal rosettes are rare. |
*Occasionally branching capillary vessels. |
*Clear cell phenotypes more common than in other locations. |
*Complete surgical resection is the best predictor. |
*CSF spread in up to 15% of tumours. |
===Posterior fossa ependymoma=== |
*Usu. 4th ventricle, less common in CPA. |
*Most frequent in children. |
*May contain tumour nodules with increased cell density. |
*Micocysts, vascular hyalinization and calcification can be present. |
*No morphologic differences between Group A and B tumours. |
*Perivascular pseudorosettes almost always present. |
*Rare papillary or tanicytic patterns. |
DDx (supratentorial and posterior fossa ependymoma): |
*[[Subependymoma]]. |
*[[Glioblastoma]] (GBM). |
*Gliomas with BCOR internal tandem duplication. |
*[[Astroblastoma]], MN1-altered. |
**Invasive border = GBM; circumscribed border of lesion = ependymoma. |
*[[Oligodendroglioma]] (Clear cell ependymoma)) |
*CNS embryonal tumour with BCOR internal tandem duplication. |
===Spinal ependymoma=== |
*Isomorphic nuclei. |
*Mitotic activity usu. very low. |
*Calcification, hemorrhage, cystic and/or metaplastic changes may be seen. |
*Most tumours show CNS grade 2 histology. |
**CNS grade 3 tumours should be examined for MYCN amplification. |
*Outcome usu. good, extent of resection is prognostic. |
DDx (spinal ependymoma): |
*[[Pilocytic astrocytoma]] (Tanycytic ependymoma) |
*Diffuse midline glioma, H3 K27-altered |
*Small cell glioblastoma (MYCN-amplified spinal ependymoma) |
===Images=== |
www: |
*[http://www.flickr.com/photos/ckrishnan/3862487821/in/photostream Ependymoma (flickr.com)]. |
*[http://www.ajnr.org/cgi/content-nw/full/27/3/488/F10 Ependymoma - ependymal rosettes (ajnr.org)]. |
*[http://path.upmc.edu/cases/case95/micro.html Anaplastic ependymoma - case 1 (upmc.edu)]. |
*[http://path.upmc.edu/cases/case324.html Anaplastic ependymoma - case 2 (upmc.edu)]. |
<gallery> |
File:AFIP405736M-EPENDYMOMA.jpg | Ependymoma smear. (AFIP) |
File:AFIP405715M-EPENDYMOMA.jpg | Perivascular pseudorosettes in a ependymoma. (AFIP) |
Image:Ependymoma_intermed_mag.jpg | Ependymoma - intermed. mag. (WC) |
Image:Ependymoma_low_intermed_mag.jpg | Ependymoma - low mag. (WC) |
File:Ependymoma_H%26E.jpg | Ependymoma - high mag. (WC/Sbrandner) |
File:Ependymoma_true_ependymal_rosettes_and_pseudorosettes.jpg | True ependymal and pseudorosettes in a ependymoma. (WC/jensflorian) |
File:Ependymal_linings_ependymoma_HE.jpg | Ependymal linings in a ependymoma. (WC/jensflorian) |
File:Ependymoma_GFAP.jpg| GFAP IHC in a ependymoma. (WC/Sbrandner) |
File:EMA_ependymoma_periluminal.jpg | Periluminal EMA positivity in a ependymoma. (WC/jensflorian) |
File:Ependymoma_EMA.jpg | Dot-like EMA immunreactivity n a ependymoma. (WC/Marvin101) |
File:Tanycytic ependymoma HE.jpg | Tanycytic morphology in ependymoma must not confused with [[pilocytic astrocytoma]]. (WC/jensflorian) |
File:Tanicytic_ependymoma_x10.jpg | Tanycytic morphology in ependymoma - low mag. (WC/jensflorian) |
File:Papillary_Ependymoma.jpg | Papillary morphology in ependymoma - low mag. (WC/jensflorian) |
File:Papillary_ependymoma_HE_x40.jpg | Papillary morphology in ependymoma - intermed. mag. (WC/jensflorian) |
File:Clear_cell_ependymoma_HE.jpg | Clear cell morphology in ependymoma may mimic [[oligodendroglioma]]. (WC/jensflorian) |
File:HE_anaplastic_epedymomas_mitoses_pleomorphism.jpg | Brisk mitotic activity in a anaplastic ependymoma. (WC/jensflorian) |
File:Cartilaginous metaplasia ependymoma.jpg|Metaplastic transformation in an anaplastic ependymoma. (WC/jensflorian) |
File:Ependymoma_L1CAM_IHC.jpg | L1CAM immunohistochemistry indicates presence of ZFTA-fusion. |
File:Ependymoma_NFkappaB_IHC.jpg | Nuclear NFkappaB IHC indicates presence of ZFTA-fusion. |
</gallery> |
===Grading=== |
Easy: |
*Subependymoma = CNS WHO grade 1. |
*Myxopapillary ependymoma = CNS WHO grade 2. |
Not so easy: |
All other ependymomas: WHO CNS Grade 2 vs. Grade 3 depends on: |
*Cellular density. |
*Mitoses (no clear cut-off). |
*Necrosis (not prognostic). |
*Microvascular proliferation. |
*Poor interobserver reliability<ref>{{Cite journal | last1 = Ellison | first1 = DW. | last2 = Kocak | first2 = M. | last3 = Figarella-Branger | first3 = D. | last4 = Felice | first4 = G. | last5 = Catherine | first5 = G. | last6 = Pietsch | first6 = T. | last7 = Frappaz | first7 = D. | last8 = Massimino | first8 = M. ... |
Notes: |
*Many tumours fall between grade 2 and grade 3. |
*Rare cases with sarcomatous or cartilaginous components.<ref>{{Cite journal | last1 = Vajtai | first1 = I. | last2 = Kuhlen | first2 = D. | last3 = Kappeler | first3 = A. | last4 = Mariani | first4 = L. | last5 = Zimmermann | first5 = A. | last6 = Paulus | first6 = W. | title = Rapid spontaneous malignant progression... |
==IHC== |
*Reticulin-ve. |
*GFAP+ve. |
*MIB1 (usu low). |
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