Difference between revisions of "Sarcoma with CIC-rearrangement"

From Libre Pathology
Jump to navigation Jump to search
(create)
 
Line 1: Line 1:
'''Undifferentiated small blue round cell sarcoma with t(4;19)''', is a rare [[malignant]] [[small round cell tumour]].<ref name=pmid23887164>{{Cite journal  | last1 = Choi | first1 = EY. | last2 = Thomas | first2 = DG. | last3 = McHugh | first3 = JB. | last4 = Patel | first4 = RM. | last5 = Roulston | first5 = D. | last6 = Schuetze | first6 = SM. | last7 = Chugh | first7 = R. | last8 = Biermann | first8 = JS. | last9 = Lucas | first9 = DR. | title = Undifferentiated small round cell sarcoma with t(4;19)(q35;q13.1) CIC-DUX4 fusion: a novel highly aggressive soft tissue tumor with distinctive histopathology. | journal = Am J Surg Pathol | volume = 37 | issue = 9 | pages = 1379-86 | month = Sep | year = 2013 | doi = 10.1097/PAS.0b013e318297a57d | PMID = 23887164 }}</ref>
'''Undifferentiated small blue round cell sarcoma with t(4;19)''', is a rare [[malignant]] [[small round cell tumour]].<ref name=pmid23887164>{{Cite journal  | last1 = Choi | first1 = EY. | last2 = Thomas | first2 = DG. | last3 = McHugh | first3 = JB. | last4 = Patel | first4 = RM. | last5 = Roulston | first5 = D. | last6 = Schuetze | first6 = SM. | last7 = Chugh | first7 = R. | last8 = Biermann | first8 = JS. | last9 = Lucas | first9 = DR. | title = Undifferentiated small round cell sarcoma with t(4;19)(q35;q13.1) CIC-DUX4 fusion: a novel highly aggressive soft tissue tumor with distinctive histopathology. | journal = Am J Surg Pathol | volume = 37 | issue = 9 | pages = 1379-86 | month = Sep | year = 2013 | doi = 10.1097/PAS.0b013e318297a57d | PMID = 23887164 }}</ref>


It is also known as '''CIC-DUX sarcoma'''.
It is also known as '''CIC-DUX sarcoma'''. ''CIC-DUX'' is pronounced "six ducks"


==General==
==General==
Line 7: Line 7:
*Young - typically 20s.
*Young - typically 20s.
*Female>male.
*Female>male.
*Aggressive course.


==Microscopic==
==Microscopic==

Revision as of 00:27, 25 March 2015

Undifferentiated small blue round cell sarcoma with t(4;19), is a rare malignant small round cell tumour.[1]

It is also known as CIC-DUX sarcoma. CIC-DUX is pronounced "six ducks"

General

  • Rare.
  • Young - typically 20s.
  • Female>male.
  • Aggressive course.

Microscopic

Features:

DDx:

IHC

  • CD99 +ve/-ve
    • Not strong and membranous.
  • WT-1 +ve.
  • S-100 -ve.
  • Desmin =ve.
  • Myogenin -ve.
  • ERG +ve.
  • FLI-1 +ve.

Molecular

  • t(4;19)(q35;q13.1).[2]
    • CIC-DUX4 fusion.
  • EWSR1 FISH negative.

See also

References

  1. Choi, EY.; Thomas, DG.; McHugh, JB.; Patel, RM.; Roulston, D.; Schuetze, SM.; Chugh, R.; Biermann, JS. et al. (Sep 2013). "Undifferentiated small round cell sarcoma with t(4;19)(q35;q13.1) CIC-DUX4 fusion: a novel highly aggressive soft tissue tumor with distinctive histopathology.". Am J Surg Pathol 37 (9): 1379-86. doi:10.1097/PAS.0b013e318297a57d. PMID 23887164.
  2. Italiano, A.; Sung, YS.; Zhang, L.; Singer, S.; Maki, RG.; Coindre, JM.; Antonescu, CR. (Mar 2012). "High prevalence of CIC fusion with double-homeobox (DUX4) transcription factors in EWSR1-negative undifferentiated small blue round cell sarcomas.". Genes Chromosomes Cancer 51 (3): 207-18. doi:10.1002/gcc.20945. PMID 22072439.