Difference between revisions of "Atypical teratoid/rhabdoid tumour"

From Libre Pathology
Jump to navigation Jump to search
m
Line 69: Line 69:
*BAF-47 -ve ([[AKA]] ''INI1'', [[AKA]] ''SMARCB1'' - the HGNC symbol<ref name=omim601607>{{OMIM|601607}}</ref>) - virtually diagnostic (4/4 cases<ref name=pmid19212771/>).
*BAF-47 -ve ([[AKA]] ''INI1'', [[AKA]] ''SMARCB1'' - the HGNC symbol<ref name=omim601607>{{OMIM|601607}}</ref>) - virtually diagnostic (4/4 cases<ref name=pmid19212771/>).
**Endothelial cells +ve control.
**Endothelial cells +ve control.
*S-100 +ve (4/4 cases<ref name=pmid19212771>{{Cite journal  | last1 = Ertan | first1 = Y. | last2 = Sezak | first2 = M. | last3 = Turhan | first3 = T. | last4 = Kantar | first4 = M. | last5 = Erşahin | first5 = Y. | last6 = Mutluer | first6 = S. | last7 = Vergin | first7 = C. | last8 = Oniz | first8 = H. | last9 = Akalin | first9 = T. | title = Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases. | journal = Childs Nerv Syst | volume = 25 | issue = 6 | pages = 707-11 | month = Jun | year = 2009 | doi = 10.1007/s00381-009-0811-0 | PMID = 19212771 }}</ref>.
*S-100 +ve (4/4 cases<ref name=pmid19212771>{{Cite journal  | last1 = Ertan | first1 = Y. | last2 = Sezak | first2 = M. | last3 = Turhan | first3 = T. | last4 = Kantar | first4 = M. | last5 = Erşahin | first5 = Y. | last6 = Mutluer | first6 = S. | last7 = Vergin | first7 = C. | last8 = Oniz | first8 = H. | last9 = Akalin | first9 = T. | title = Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases. | journal = Childs Nerv Syst | volume = 25 | issue = 6 | pages = 707-11 | month = Jun | year = 2009 | doi = 10.1007/s00381-009-0811-0 | PMID = 19212771 }}</ref>).
**Few other brain tumours express it.
**Few other brain tumours express it.
*Vimentin +ve - perinuclear condensation (4/4 cases<ref name=pmid19212771/>).
*Vimentin +ve - perinuclear condensation (4/4 cases<ref name=pmid19212771/>).

Revision as of 18:18, 18 March 2015

Atypical teratoid/rhabdoid tumour
Diagnosis in short

AT/RT. H&E stain.
LM cellular tumour with small round cells usu. with a prominent nucleolus, rhabdoid cells (eosinophilic granular cytoplasm + eccentric nucleus), mitoses. +/-necrosis (common)
LM DDx primitive neuroectodermal tumour (PNET), medulloblastoma, diffuse astrocytoma, choroid plexus carcinoma,embryonal carcinoma
IHC INI1 -ve, S-100 +ve, EMA +ve, SMA +ve
Site CNS - typically supratentorial
Clinical history usu. <3 years olds, occasionally adults
Prevalence uncommon - esp. in adults
Prognosis very poor

Atypical teratoid/rhabdoid tumour, abbreviated AT/RT, is malignant tumour usually found supratentorially.

It may be written atypical teratoid rhabdoid tumour (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 usually <3 years old, uncommon 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:

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 (4/4 cases[3]).
    • Endothelial cells +ve control.
  • S-100 +ve (4/4 cases[3]).
    • Few other brain tumours express it.
  • Vimentin +ve - perinuclear condensation (4/4 cases[3]).

Others:

  • GFAP +ve (focal - in tumour cells).
  • EMA +ve - patchy cytoplasmic (4/4 cases[3]).
  • Smooth muscle actin +ve.(4/4 cases[3]).
  • Cytokeratin +ve.[citation needed]

See also

References

  1. ↑ 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.
  2. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601607
  3. ↑ 3.0 3.1 3.2 3.3 3.4 Ertan, Y.; Sezak, M.; Turhan, T.; Kantar, M.; Erşahin, Y.; Mutluer, S.; Vergin, C.; Oniz, H. et al. (Jun 2009). "Atypical teratoid/rhabdoid tumor of the central nervous system: clinicopathologic and immunohistochemical features of four cases.". Childs Nerv Syst 25 (6): 707-11. doi:10.1007/s00381-009-0811-0. PMID 19212771.
Retrieved from "https://librepathology.org/w/index.php?title=Atypical_teratoid/rhabdoid_tumour&oldid=36876"