Difference between revisions of "Atypical teratoid/rhabdoid tumour"
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'''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]] | |||
Revision as of 03:15, 17 February 2014
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
Rhabdoid tumour cell. (WC)
AT/RT. (WC)
AT/RT -ve for INI1. (WC)
www:
IHC
- BAF-47 -ve (AKA INI1, AKA SMARCB1 - the HGNC symbol[1]) - 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.