Difference between revisions of "Atypical teratoid/rhabdoid tumour"
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==General== | ==General== | ||
*Usually supratentorial, occasionally in posterior fossa, case reports of spinal cord. | *Usually supratentorial, occasionally in posterior fossa, case reports of spinal cord. | ||
*Individuals usu. <3 years old, very rare in adults.<ref name=pmid24477665>{{Cite journal | last1 = Kanoto | first1 = M. | last2 = Toyoguchi | first2 = Y. | last3 = Hosoya | first3 = T. | last4 = Kuchiki | first4 = M. | last5 = Sugai | first5 = Y. | title = Radiological Image Features of the Atypical Teratoid/Rhabdoid Tumor in Adults: A Systematic Review. | journal = Clin Neuroradiol | volume = | issue = | pages = | month = Jan | year = 2014 | doi = 10.1007/s00062-013-0282-2 | PMID = 24477665 }}</ref> | |||
*Prognosis very poor.<ref name=pmid24477665/> | |||
==Microscopic== | ==Microscopic== | ||
Revision as of 03:20, 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.
- Individuals usu. <3 years old, very rare in adults.[1]
- Prognosis very poor.[1]
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[2]) - 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
References
- ↑ 1.0 1.1 Kanoto, M.; Toyoguchi, Y.; Hosoya, T.; Kuchiki, M.; Sugai, Y. (Jan 2014). "Radiological Image Features of the Atypical Teratoid/Rhabdoid Tumor in Adults: A Systematic Review.". Clin Neuroradiol. doi:10.1007/s00062-013-0282-2. PMID 24477665.
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601607