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Pleomorphic xanthoastrocytoma (view source)
Revision as of 12:38, 11 October 2019
, 12:38, 11 October 2019→Images: APXA
Jensflorian (talk | contribs) (+update +pictures from current case) |
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*Associated with seizures. | *Associated with seizures. | ||
*Moderately aggressive (WHO Grade II).<ref name=pmid11465399>{{Cite journal | last1 = Fouladi | first1 = M. | last2 = Jenkins | first2 = J. | last3 = Burger | first3 = P. | last4 = Langston | first4 = J. | last5 = Merchant | first5 = T. | last6 = Heideman | first6 = R. | last7 = Thompson | first7 = S. | last8 = Sanford | first8 = A. | last9 = Kun | first9 = L. | title = Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. | journal = Neuro Oncol | volume = 3 | issue = 3 | pages = 184-92 | month = Jul | year = 2001 | doi = | PMID = 11465399 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1920613/pdf/11465399.pdf}}</ref> | *Moderately aggressive (WHO Grade II).<ref name=pmid11465399>{{Cite journal | last1 = Fouladi | first1 = M. | last2 = Jenkins | first2 = J. | last3 = Burger | first3 = P. | last4 = Langston | first4 = J. | last5 = Merchant | first5 = T. | last6 = Heideman | first6 = R. | last7 = Thompson | first7 = S. | last8 = Sanford | first8 = A. | last9 = Kun | first9 = L. | title = Pleomorphic xanthoastrocytoma: favorable outcome after complete surgical resection. | journal = Neuro Oncol | volume = 3 | issue = 3 | pages = 184-92 | month = Jul | year = 2001 | doi = | PMID = 11465399 | URL = http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1920613/pdf/11465399.pdf}}</ref> | ||
*Anaplastic PXA (grade III) - rare. <ref>{{Cite journal | last1 = Louis | first1 = DN. | last2 = Perry | first2 = A. | last3 = Reifenberger | first3 = G. | last4 = von Deimling | first4 = A. | last5 = Figarella-Branger | first5 = D. | last6 = Cavenee | first6 = WK. | last7 = Ohgaki | first7 = H. | last8 = Wiestler | first8 = OD. | last9 = Kleihues | first9 = P. | title = The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 803-20 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1545-1 | PMID = 27157931 }}</ref> | |||
*ICD-O: 9424/3. | *ICD-O: 9424/3. | ||
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*Eosinophilic granular bodies - very common.<ref name=pmid11465399/> | *Eosinophilic granular bodies - very common.<ref name=pmid11465399/> | ||
*Inflammatory cells (lymophocytic perivascular cuffs). | *Inflammatory cells (lymophocytic perivascular cuffs). | ||
*Mitotic activity is low | |||
==Grading== | |||
* Grade II: Mitotic activity is low, no necrosis. | |||
* Grade III anaplastic PXA: more than 5/10 HPF, may have necrosis. | |||
Notes: | Notes: | ||
* | Anaplastic PXA was introduced in the WHO2016 revision as a distinct entity. | ||
* | In the past, the tumor was called ''PXA with anaplastic features''. | ||
DDx: | |||
*[[Subependymal giant cell astrocytoma]]. | |||
*[[Ganglioglioma]]. | |||
*[[Glioblastoma]]. | |||
===Images=== | ===Images=== | ||
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File:PXA HE xanthomatous cells.jpg| Focal xanthomatous cells as in the name PXA. HE, intermed magnification.(WC/jensflorian) | File:PXA HE xanthomatous cells.jpg| Focal xanthomatous cells as in the name PXA. HE, intermed magnification.(WC/jensflorian) | ||
File:PXA HE X10.jpg | Perivascular lymphocytic cuffs are not uncommon. HE, intermed-low magnification.(WC/jensflorian) | File:PXA HE X10.jpg | Perivascular lymphocytic cuffs are not uncommon. HE, intermed-low magnification.(WC/jensflorian) | ||
File:PXA HE x20.jpg | | File:PXA HE x20.jpg | Eosinophilc granular bodies in a PXA. HE, intermed magnification.(WC/jensflorian) | ||
File:PXA Gomori Reticulin Stain.jpg | A delicate | File:PXA Gomori Reticulin Stain.jpg | A delicate meshwork of retiulin fibers in PXA. Gomori, intermed magnification.(WC/jensflorian) | ||
File:PXA GFAP IHC.jpg | GFAP IHC is often heterogenous in PXA. Intermed magnification.(WC/jensflorian) | File:PXA GFAP IHC.jpg | GFAP IHC is often heterogenous in PXA. Intermed magnification.(WC/jensflorian) | ||
File:Anaplastic_pxa_histology.jpg| HE of anaplastic PXA. | |||
</gallery> | </gallery> | ||
www: | www: | ||
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* BRAF V600E mutation in 2/3 of the cases<ref>{{Cite journal | last1 = Schindler | first1 = G. | last2 = Capper | first2 = D. | last3 = Meyer | first3 = J. | last4 = Janzarik | first4 = W. | last5 = Omran | first5 = H. | last6 = Herold-Mende | first6 = C. | last7 = Schmieder | first7 = K. | last8 = Wesseling | first8 = P. | last9 = Mawrin | first9 = C. | title = Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. | journal = Acta Neuropathol | volume = 121 | issue = 3 | pages = 397-405 | month = Mar | year = 2011 | doi = 10.1007/s00401-011-0802-6 | PMID = 21274720 }}</ref> | * BRAF V600E mutation in 2/3 of the cases<ref>{{Cite journal | last1 = Schindler | first1 = G. | last2 = Capper | first2 = D. | last3 = Meyer | first3 = J. | last4 = Janzarik | first4 = W. | last5 = Omran | first5 = H. | last6 = Herold-Mende | first6 = C. | last7 = Schmieder | first7 = K. | last8 = Wesseling | first8 = P. | last9 = Mawrin | first9 = C. | title = Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. | journal = Acta Neuropathol | volume = 121 | issue = 3 | pages = 397-405 | month = Mar | year = 2011 | doi = 10.1007/s00401-011-0802-6 | PMID = 21274720 }}</ref> | ||
**(mostly in temporal, reticulin-fiber rich tumors)<ref>{{Cite journal | last1 = Koelsche | first1 = C. | last2 = Sahm | first2 = F. | last3 = Wöhrer | first3 = A. | last4 = Jeibmann | first4 = A. | last5 = Schittenhelm | first5 = J. | last6 = Kohlhof | first6 = P. | last7 = Preusser | first7 = M. | last8 = Romeike | first8 = B. | last9 = Dohmen-Scheufler | first9 = H. | title = BRAF-mutated pleomorphic xanthoastrocytoma is associated with temporal location, reticulin fiber deposition and CD34 expression. | journal = Brain Pathol | volume = 24 | issue = 3 | pages = 221-9 | month = Apr | year = 2014 | doi = 10.1111/bpa.12111 | PMID = 24345274 }}</ref> | **(mostly in temporal, reticulin-fiber rich tumors)<ref>{{Cite journal | last1 = Koelsche | first1 = C. | last2 = Sahm | first2 = F. | last3 = Wöhrer | first3 = A. | last4 = Jeibmann | first4 = A. | last5 = Schittenhelm | first5 = J. | last6 = Kohlhof | first6 = P. | last7 = Preusser | first7 = M. | last8 = Romeike | first8 = B. | last9 = Dohmen-Scheufler | first9 = H. | title = BRAF-mutated pleomorphic xanthoastrocytoma is associated with temporal location, reticulin fiber deposition and CD34 expression. | journal = Brain Pathol | volume = 24 | issue = 3 | pages = 221-9 | month = Apr | year = 2014 | doi = 10.1111/bpa.12111 | PMID = 24345274 }}</ref> | ||
==See also== | ==See also== | ||