Pleomorphic xanthoastrocytoma: Difference between revisions

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'''Pleomorphic xanthoastrocytoma''', abbreviated '''PXA''', is [[neuropathology tumour]] classically associated with seizures.

{{ Infobox diagnosis

| Name = {{PAGENAME}}

| Image = Pleo xantho.jpg

| Width =

| Caption = Pleomorphic xanthoastrocytoma.

| Synonyms =

| Micro = marked nuclear atypia, eosinophilic granular bodies - very common, inflammation (chronic), no necrosis

| Subtypes =

| LMDDx =

| Stains =

| IHC =

| EM =

| Molecular =

| IF =

| Gross =

| Grossing =

| Site = [[brain]] - typical temporal lobe

| Assdx =

| Syndromes =

| Clinicalhx = seizure, children & young adults

| Signs =

| Symptoms =

| Prevalence =

| Bloodwork =

| Rads =

| Endoscopy =

| Prognosis =

| Other =

| ClinDDx =

| Tx =

}}

'''Pleomorphic xanthoastrocytoma''', abbreviated '''PXA''', is [[neuropathology tumour]] classically associated with seizures in children.

==General==

Features:

*Rare (less than 1% of all astrocytic tumors).

*Classically in the temporal lobe in children and young adults.

*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>

*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.

==Gross==

*Temporal lobe - classic.

*Usually assoc. with the leptomeninges,<ref name=pmid11465399/> i.e. superficial.

*Usually assoc. with the leptomeninges,<ref name=pmid11465399/> i.e. superficial (in up 96%).

File:405663-PLEOMORPHIC XANTHOASTROCYTOMA.jpg | Typical appearance is one of a superficially situated tumor, here a mural nodule, associated with an underlying cyst (AFIP)

File:Pleomorphic-xanthoastrocytoma-002.jpg | Hemorrhagic pleomorphic xanthoastrocytoma (PXA), MRI (WC/RadsWiki)

</gallery>

==Microscopic==

Features:<ref name=Ref_PBoD8_1333>{{Ref PBoD8|1333}}</ref>

*~~Marked~~ nuclear atypia.

*Fibrillary background.

*Large cells with marked nuclear atypia.

*Multinuclear cells possible.

*Reticulin meshwork.

*Lipidized cells.

*Eosinophilic granular bodies - very common.<ref name=pmid11465399/>

*~~Inflammation~~ (~~chronic~~).

*Inflammatory cells (lymophocytic perivascular cuffs).

==Grading==

* Grade II: Mitotic activity is low, no necrosis.

* Grade III anaplastic PXA: more than 5/10 HPF, may have necrosis.

Notes:

*~~No mitoses~~.

Anaplastic PXA was introduced in the WHO2016 revision as a distinct entity.

*No [[~~necrosis~~]].

In the past, the tumor was called ''PXA with anaplastic features''.

DDx:

*[[Subependymal giant cell astrocytoma]].

*[[Ganglioglioma]].

*[[Glioblastoma]].

===Images===

Image:Pleo xantho.jpg | HE smear of a PXA. (WC/AFIP)

File:PXA HE frozen high mag.jpg | HE intraoperative frozen section of PXA. (WC/jensflorian)

File:PXA HE 40x-2.jpg | The tumor is aptly named due to ist pleomorphic cells. HE, high magnification. (WC/marvin101)

File:PXA HE pleomorphic cells.jpg | The pleomorphic cells must not be confused with a higly anaplastic glioma. 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 x20.jpg | Eosinophilc granular bodies in a PXA. HE, intermed magnification.(WC/jensflorian)

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:Anaplastic_pxa_histology.jpg| HE of anaplastic PXA.

</gallery>

www:

*[http://path.upmc.edu/cases/case499.html Pleomorphic xanthoastrocytoma - several images (upmc.edu)].

*[http://path.upmc.edu/cases/case511.html Pleomorphic xanthoastrocytoma with anaplasia - another case - several images (upmc.edu)].

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==IHC==

*GFAP +ve.

*S-100 +ve.

*CD68 +ve.

*CD34 frequently.

*MAP2+ve and Synapto+ve pleomorphic cells

*MIB-1 usually low.

==Molecular==

* 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>

==See also==

@@ Line 1: / Line 1: @@
-'''Pleomorphic xanthoastrocytoma''', abbreviated '''PXA''', is [[neuropathology tumour]] classically associated with seizures.
+{{ Infobox diagnosis
+| Name       = {{PAGENAME}}
+| Image      = Pleo xantho.jpg
+| Width      =
+| Caption    = Pleomorphic xanthoastrocytoma.
+| Synonyms   =
+| Micro      = marked nuclear atypia, eosinophilic granular bodies - very common, inflammation (chronic), no necrosis
+| Subtypes   =
+| LMDDx      =
+| Stains     =
+| IHC        =
+| EM         =
+| Molecular  =
+| IF         =
+| Gross      =
+| Grossing   =
+| Site       = [[brain]] - typical temporal lobe
+| Assdx      =
+| Syndromes  =
+| Clinicalhx = seizure, children & young adults
+| Signs      =
+| Symptoms   =
+| Prevalence =
+| Bloodwork  =
+| Rads       =
+| Endoscopy  =
+| Prognosis  =
+| Other      =
+| ClinDDx    =
+| Tx         =
+}}
+'''Pleomorphic xanthoastrocytoma''', abbreviated '''PXA''', is [[neuropathology tumour]] classically associated with seizures in children.
 ==General==
 Features:
+*Rare (less than 1% of all astrocytic tumors).
 *Classically in the temporal lobe in children and young adults.
 *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>
+*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.
 ==Gross==
 *Temporal lobe - classic.
-*Usually assoc. with the leptomeninges,<ref name=pmid11465399/> i.e. superficial.
+*Usually assoc. with the leptomeninges,<ref name=pmid11465399/> i.e. superficial (in up 96%).
+<gallery>
+File:405663-PLEOMORPHIC XANTHOASTROCYTOMA.jpg |  Typical appearance is one of a superficially situated tumor, here a mural nodule, associated with an underlying cyst (AFIP)
+File:Pleomorphic-xanthoastrocytoma-002.jpg | Hemorrhagic pleomorphic xanthoastrocytoma (PXA), MRI (WC/RadsWiki)
+</gallery>
 ==Microscopic==
 Features:<ref name=Ref_PBoD8_1333>{{Ref PBoD8|1333}}</ref>
-*Marked nuclear atypia.
+*Fibrillary background.
+*Large cells with marked nuclear atypia.
+*Multinuclear cells possible.
+*Reticulin meshwork.
+*Lipidized cells.
 *Eosinophilic granular bodies - very common.<ref name=pmid11465399/>
-*Inflammation (chronic).
+*Inflammatory cells (lymophocytic perivascular cuffs).
+==Grading==
+* Grade II: Mitotic activity is low, no necrosis.
+* Grade III anaplastic PXA: more than 5/10 HPF, may have necrosis.
 Notes:
-*No mitoses.
+Anaplastic PXA was introduced in the WHO2016 revision as a distinct entity.
-*No [[necrosis]].
+In the past, the tumor was called ''PXA with anaplastic features''.
+DDx:
+*[[Subependymal giant cell astrocytoma]].
+*[[Ganglioglioma]].
+*[[Glioblastoma]].
 ===Images===
+<gallery>
+Image:Pleo xantho.jpg | HE smear of a PXA. (WC/AFIP)
+File:PXA HE frozen high mag.jpg | HE intraoperative frozen section of PXA. (WC/jensflorian)
+File:PXA HE 40x-2.jpg | The tumor is aptly named due to ist pleomorphic cells. HE, high magnification. (WC/marvin101)
+File:PXA HE pleomorphic cells.jpg | The pleomorphic cells must not be confused with a higly anaplastic glioma. 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 x20.jpg | Eosinophilc granular bodies in a PXA. HE, intermed magnification.(WC/jensflorian)
+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:Anaplastic_pxa_histology.jpg| HE of anaplastic PXA.
+</gallery>
+www:
 *[http://path.upmc.edu/cases/case499.html Pleomorphic xanthoastrocytoma - several images (upmc.edu)].
 *[http://path.upmc.edu/cases/case511.html Pleomorphic xanthoastrocytoma with anaplasia - another case - several images (upmc.edu)].
@@ Line 35: / Line 102: @@
 ==IHC==
 *GFAP +ve.
+*S-100 +ve.
 *CD68 +ve.
+*CD34 frequently.
+*MAP2+ve and Synapto+ve pleomorphic cells
+*MIB-1 usually low.
+==Molecular==
+* 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>
 ==See also==

Pleomorphic xanthoastrocytoma: Difference between revisions

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