Difference between revisions of "Neuropathology tumours"

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==Medulloblastoma==
==Medulloblastoma==
===General===
{{Main|Medulloblastoma}}
*Mostly paediatric population.
*Tumour of cerebellum - key feature.
*May be seen as a component of [[nevoid basal cell carcinoma syndrome]] (NBCCS).
*Morphologically identical supratentorial tumours are called [[primitive neuroectodermal tumour]] (PNET).
**Gene: ''patched'' (abbreviated ''PTCH1'').<ref name=omim601309>{{OMIM|601309}}</ref>
*Commonly spread via cerebrospinal fluid (CSF).<ref>{{Ref APBR|424 Q34}}</ref>
**May be detected in [[CSF cytopathology]] specimens.
 
===Gross===
*Location: cerebellum - '''key feature'''.
**Morphologically identical supratentorial tumours are called ''[[primitive neuroectodermal tumour]]'' (PNET).
 
===Microscopic===
Features:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm]. Accessed on: 26 October 2010.</ref>
*[[Small round cell tumour]].
*Homer-Wright [[rosette]]s:
**Rosette with a meshwork of fibers (neuropil) at the centre.<ref>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref>
 
Images:
*[http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm Medulloblastoma (ouhsc.edu)].
*[http://path.upmc.edu/cases/case667.html Medulloblastoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case685.html Medulloblastoma with rhabdomyoblastic differentiation - several images (upmc.edu)].
 
DDx:
*[[Small round cell tumours]].
 
====Subtypes====
*Classic medulloblastoma (~85% of all medulloblastomas).
*Variants of medulloblastoma (~15% of all medulloblastomas together):
*#Anaplastic variant.
*#Large cell variant.
*#Desmoplastic/nodular medulloblastoma (DNMB).
*#Medulloblastoma with extensive nodularity (MBEN).
 
Notes:
*Prognosis:<ref name=pmid18841049>{{cite journal |author=Gulino A, Arcella A, Giangaspero F |title=Pathological and molecular heterogeneity of medulloblastoma |journal=Curr Opin Oncol |volume=20 |issue=6 |pages=668–75 |year=2008 |month=November |pmid=18841049 |doi=10.1097/CCO.0b013e32831369f4 |url=}}</ref><ref name=pmid20940197>{{cite journal |author=Rutkowski S, von Hoff K, Emser A, ''et al.'' |title=Survival and Prognostic Factors of Early Childhood Medulloblastoma: An International Meta-Analysis |journal=J Clin Oncol |volume=28 |issue=33 |pages=4961–4968 |year=2010 |month=November |pmid=20940197 |doi=10.1200/JCO.2010.30.2299 |url=}}</ref> DNMB & MBEN > classic > anaplastic variant, large cell variant.
 
=====Anaplastic variant=====
Features:
*Larger cells.
*Severe anaplasia.
*Polygonal cells.


==Primitive neuroectodermal tumour==  
==Primitive neuroectodermal tumour==  
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