Difference between revisions of "Pilomyxoid astrocytoma"

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#redirect [[Neuropathology_tumours#Pilomyxoid_astrocytoma]]
'''Pilomyxoid astrocytoma''' is a [[neuropathology tumour]] related to [[pilocytic astrocytoma]].
 
==General==
Features:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*A variant of ''pilocytic astrocytoma''.
**Some have suggested it is a unique entity.<ref name=pmid16048293>{{cite journal |author=Komotar RJ, Mocco J, Jones JE, ''et al.'' |title=Pilomyxoid astrocytoma: diagnosis, prognosis, and management |journal=Neurosurg Focus |volume=18 |issue=6A |pages=E7 |year=2005 |month=June |pmid=16048293 |doi= |url=}}</ref>
**Some say the outcome is less good than conventional pilocytic astrocytoma.<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>
**Tumors may mature to conventional PA over time.<ref>{{Cite journal  | last1 = Kleinschmidt-DeMasters | first1 = BK. | last2 = Donson | first2 = AM. | last3 = Vogel | first3 = H. | last4 = Foreman | first4 = NK. | title = Pilomyxoid Astrocytoma (PMA) Shows Significant Differences in Gene Expression vs. Pilocytic Astrocytoma (PA) and Variable Tendency Toward Maturation to PA. | journal = Brain Pathol | volume = 25 | issue = 4 | pages = 429-40 | month = Jul | year = 2015 | doi = 10.1111/bpa.12239 | PMID = 25521223 }}</ref>
*Childhood or adolescence.
*ICD-O: 9425/3
 
Grading:
*Currently no WHO grade assigned.<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>
** From 2007-2016 the tumor was ''WHO Grade II'' by definition.<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref> Grading was dropped until further studies clarify the true behaviour.
 
==Gross==
Features:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*Classically - hypothalamic location/suprasellar location; may involve the [[sella turcica]].<ref name=pmid19766001>{{cite journal |author=Alimohamadi M, Bidabadi MS, Ayan Z, Ketabchi E, Amirjamshidi A |title=Pilomyxoid astrocytoma with involvement of the sella turcica in an adolescent |journal=J Clin Neurosci |volume=16 |issue=12 |pages=1648–9 |year=2009 |month=December |pmid=19766001 |doi=10.1016/j.jocn.2009.01.035 |url=}}</ref>
*Solid.
*Well-circumscribed.
 
==Microscopic==
Features:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*Consists of small round/ovoid bland cells in a [[myxoid stroma]].
*Hair-like fibres ~ 1 micrometer.
**Often difficult to appreciate on standard (H&E) histologic sections.
*Usually angiocentric (surround blood vessel) - '''key feature'''.
 
Notes:<ref name=Ref_PSNP86>{{Ref PSNP|86}}</ref>
*Rosenthal fibres are absent - '''key negative'''.
*Monophasic (unlike classical pilocytic astrocytomas) - '''key negative'''.
*May rarely have eosinophilic granular bodies.
 
===Images===
<gallery>
Image:Pilomyxoid Astrocytoma HE20x.jpg | Pilomyxoid astrocytoma. (WC/Marvin 101)
File:Pilomyxoid astrocytoma 03.jpg | Pilomyxoid astrocytoma. (WC)
File:Pilomyxoid astrocytoma 01.jpg | Pilomyxoid astrocytoma - Alcian blue (WC)
</gallery>
 
==See also==
*[[Neuropathology tumours]].
*[[Pilocytic astrocytoma]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Neuropathology tumours]]
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