Difference between revisions of "Atypical teratoid/rhabdoid tumour"

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#redirect [[Neuropathology_tumours#Atypical_teratoid.2Frhabdoid_tumour]]
'''Atypical teratoid/rhabdoid tumour''', abbreviated '''AT/RT''', is malignant tumour usually found supratentorially.
 
It may be written '''atypical teratoid rhabdoid tumour''', i.e. without the forward slash (abbreviated '''ATRT''', or ''atypical teratoid-rhabdoid tumour'' (abbreviated '''AT-RT''').
 
It should '''not''' be confused with the ''[[extrarenal malignant rhabdoid tumour]]''.
 
==General==
*Usually supratentorial, occasionally in posterior fossa, case reports of spinal cord.
 
==Microscopic==
Features:
*Cellular.
*Small round cells usu. with a prominent nucleolus.
*Rhabdoid cells.
**Cells with eosinophilic granular cytoplasm + eccentric nucleus.
*Mitoses.
*+/-[[Necrosis]] (common).
 
DDx:
*[[Primitive neuroectodermal tumour]] (PNET).
*[[Medulloblastoma]].
*[[Diffuse astrocytoma]].
*[[Choroid plexus carcinoma]].
*[[Embryonal carcinoma]].
 
===Images===
<gallery>
Image:Rhabdoidtumourcell.jpg | Rhabdoid tumour cell. (WC)
Image:ATRT-HE-Overview.jpg | AT/RT. (WC)
Image:ATRT-INI1.jpg | AT/RT -ve for INI1. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case99/micro.html AR/RT (upmc.edu)].
 
==IHC==
*BAF-47 -ve ([[AKA]] ''INI1'', [[AKA]] ''SMARCB1'' - the HGNC symbol<ref name=omim601607>{{OMIM|601607}}</ref>) - virtually diagnostic.
**Endothelial cells +ve control.
*S-100 +ve.
**Few other brain tumours express it.
*Vimentin +ve (perinuclear condensation).
 
Others:
*GFAP +ve (focal - in tumour cells).
*EMA +ve (patchy cytoplasmic).
*Smooth muscle actin +ve.
 
==See also==
*[[Neuropathology tumours]].
 
==References==
{{Reflist|2}}


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