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Difference between revisions of "Sarcoma with CIC-rearrangement"
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Sarcoma with CIC-rearrangement (view source)
Revision as of 00:57, 20 March 2019
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'''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") and '''CIC sarcoma'''.<ref>{{Cite journal | last1 = Antonescu | first1 = CR. | last2 = Owosho | first2 = AA. | last3 = Zhang | first3 = L. | last4 = Chen | first4 = S. | last5 = Deniz | first5 = K. | last6 = Huryn | first6 = JM. | last7 = Kao | first7 = YC. | last8 = Huang | first8 = SC. | last9 = Singer | first9 = S. | title = Sarcomas With CIC-rearrangements Are a Distinct Pathologic Entity With Aggressive Outcome: A Clinicopathologic and Molecular Study of 115 Cases. | journal = Am J Surg Pathol | volume = 41 | issue = 7 | pages = 941-949 | month = Jul | year = 2017 | doi = 10.1097/PAS.0000000000000846 | PMID = 28346326 }}</ref> | ||
==General== | ==General== | ||
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**Patchy cytoplasmic clearing. | **Patchy cytoplasmic clearing. | ||
**Necrosis. | **Necrosis. | ||
**Irregular spacing of cells. | |||
**+/-Big nucleoli. | |||
DDx: | DDx: | ||
*[[Ewing sarcoma]]. | *[[Ewing sarcoma]] - has regular spacing of nuclei. | ||
*Other [[Ewing-like tumours]]. | *Other [[Ewing-like tumours]]. | ||
*[[Desmoplastic small round cell tumour]]. | *[[Desmoplastic small round cell tumour]]. | ||
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==IHC== | ==IHC== | ||
*[[CD99]] +ve | *[[CD99]] +ve patchy. | ||
**Not strong and membranous. | **Not strong and membranous; [[Ewing sarcoma]] is diffuse. | ||
*WT-1 +ve. | *WT-1 +ve. | ||
*S-100 -ve. | *S-100 -ve. | ||