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CIC-rearranged sarcoma

MedGen UID:
859358
•Concept ID:
C3899764
•
Neoplastic Process
Synonym: CIC-DUX Sarcoma
SNOMED CT: CIC-rearranged sarcoma (1186889001)
 
Monarch Initiative: MONDO:0956989

Definition

CIC-rearranged sarcoma is a high-grade round cell undifferentiated sarcoma with CIC-related gene fusions. [from SNOMEDCT_US]

Recent clinical studies

Etiology

Differential cyclin-E1 expression in CIC-rearranged sarcoma.
Karabulut B, Yukruk FA, Yenidunya S, Kandemir O, Kosemehmetoglu K
Ann Diagn Pathol 2024 Oct;72:152320. Epub 2024 Apr 27 doi: 10.1016/j.anndiagpath.2024.152320. PMID: 38703529
Clinical outcomes of patients with CIC-rearranged sarcoma: a single institution retrospective analysis.
Murphy J, Resch EE, Leland C, Meyer CF, Llosa NJ, Gross JM, Pratilas CA
J Cancer Res Clin Oncol 2024 Mar 4;150(3):112. doi: 10.1007/s00432-024-05631-7. PMID: 38436779Free PMC Article
A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl).
Palmerini E, Gambarotti M, Italiano A, Nathenson MJ, Ratan R, Dileo P, Provenzano S, Jones RL, DuBois SG, Martin-Broto J, de Alava E, Baldi GG, Grignani G, Ferraresi V, Brunello A, Paoluzzi L, Bertulli R, Hindi N, Montemurro M, Rothermundt C, Cocchi S, Salguero-Aranda C, Donati D, Martin JD, Abdelhamid Ahmed AH, Mazzocca A, Carretta E, Cesari M, Pierini M, Righi A, Sbaraglia M, Laginestra MA, Scotlandi K, Dei Tos AP, Ibrahim T, Stacchiotti S, Vincenzi B
Eur J Cancer 2023 Apr;183:11-23. Epub 2023 Jan 18 doi: 10.1016/j.ejca.2023.01.003. PMID: 36791667
Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.
Yoshida A
Pathol Int 2023 Jan;73(1):12-26. Epub 2022 Dec 9 doi: 10.1111/pin.13293. PMID: 36484765Free PMC Article
Molecular classification and outcome of children with rare CNS embryonal tumors: results from St. Jude Children's Research Hospital including the multi-center SJYC07 and SJMB03 clinical trials.
Liu APY, Dhanda SK, Lin T, Sioson E, Vasilyeva A, Gudenas B, Tatevossian RG, Jia S, Neale G, Bowers DC, Hassall T, Partap S, Crawford JR, Chintagumpala M, Bouffet E, McCowage G, Broniscer A, Qaddoumi I, Armstrong G, Wright KD, Upadhyaya SA, Vinitsky A, Tinkle CL, Lucas J, Chiang J, Indelicato DJ, Sanders R, Klimo P Jr, Boop FA, Merchant TE, Ellison DW, Northcott PA, Orr BA, Zhou X, Onar-Thomas A, Gajjar A, Robinson GW
Acta Neuropathol 2022 Oct;144(4):733-746. Epub 2022 Aug 18 doi: 10.1007/s00401-022-02484-7. PMID: 35982322Free PMC Article

Diagnosis

Updates on WHO classification for small round cell tumors: Ewing sarcoma vs. everything else.
Dehner CA, Lazar AJ, Chrisinger JSA
Hum Pathol 2024 May;147:101-113. Epub 2024 Jan 26 doi: 10.1016/j.humpath.2024.01.007. PMID: 38280658
CIC-Rearranged Sarcoma.
Makise N, Yoshida A
Surg Pathol Clin 2024 Mar;17(1):141-151. Epub 2023 Aug 1 doi: 10.1016/j.path.2023.06.003. PMID: 38278603
A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl).
Palmerini E, Gambarotti M, Italiano A, Nathenson MJ, Ratan R, Dileo P, Provenzano S, Jones RL, DuBois SG, Martin-Broto J, de Alava E, Baldi GG, Grignani G, Ferraresi V, Brunello A, Paoluzzi L, Bertulli R, Hindi N, Montemurro M, Rothermundt C, Cocchi S, Salguero-Aranda C, Donati D, Martin JD, Abdelhamid Ahmed AH, Mazzocca A, Carretta E, Cesari M, Pierini M, Righi A, Sbaraglia M, Laginestra MA, Scotlandi K, Dei Tos AP, Ibrahim T, Stacchiotti S, Vincenzi B
Eur J Cancer 2023 Apr;183:11-23. Epub 2023 Jan 18 doi: 10.1016/j.ejca.2023.01.003. PMID: 36791667
The 2020 WHO Classification: What's New in Soft Tissue Tumor Pathology?
Kallen ME, Hornick JL
Am J Surg Pathol 2021 Jan;45(1):e1-e23. doi: 10.1097/PAS.0000000000001552. PMID: 32796172
Round Cell Sarcomas: Newcomers and Diagnostic Approaches.
Nagy A, Somers GR
Surg Pathol Clin 2020 Dec;13(4):763-782. Epub 2020 Oct 9 doi: 10.1016/j.path.2020.08.004. PMID: 33183732

Therapy

Molecular classification and outcome of children with rare CNS embryonal tumors: results from St. Jude Children's Research Hospital including the multi-center SJYC07 and SJMB03 clinical trials.
Liu APY, Dhanda SK, Lin T, Sioson E, Vasilyeva A, Gudenas B, Tatevossian RG, Jia S, Neale G, Bowers DC, Hassall T, Partap S, Crawford JR, Chintagumpala M, Bouffet E, McCowage G, Broniscer A, Qaddoumi I, Armstrong G, Wright KD, Upadhyaya SA, Vinitsky A, Tinkle CL, Lucas J, Chiang J, Indelicato DJ, Sanders R, Klimo P Jr, Boop FA, Merchant TE, Ellison DW, Northcott PA, Orr BA, Zhou X, Onar-Thomas A, Gajjar A, Robinson GW
Acta Neuropathol 2022 Oct;144(4):733-746. Epub 2022 Aug 18 doi: 10.1007/s00401-022-02484-7. PMID: 35982322Free PMC Article
Plocabulin, a Novel Tubulin Inhibitor, Has Potent Antitumour Activity in Patient-Derived Xenograft Models of Soft Tissue Sarcoma.
Wang Y, Wozniak A, Cornillie J, Avilés P, Debiec-Rychter M, Sciot R, Schöffski P
Int J Mol Sci 2022 Jul 5;23(13) doi: 10.3390/ijms23137454. PMID: 35806460Free PMC Article
Practical use and utility of fluorescence in situ hybridization in the pathological diagnosis of soft tissue and bone tumors.
Sugita S, Hasegawa T
J Orthop Sci 2017 Jul;22(4):601-612. Epub 2017 Mar 6 doi: 10.1016/j.jos.2017.02.004. PMID: 28274512

Prognosis

Updates on WHO classification for small round cell tumors: Ewing sarcoma vs. everything else.
Dehner CA, Lazar AJ, Chrisinger JSA
Hum Pathol 2024 May;147:101-113. Epub 2024 Jan 26 doi: 10.1016/j.humpath.2024.01.007. PMID: 38280658
A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl).
Palmerini E, Gambarotti M, Italiano A, Nathenson MJ, Ratan R, Dileo P, Provenzano S, Jones RL, DuBois SG, Martin-Broto J, de Alava E, Baldi GG, Grignani G, Ferraresi V, Brunello A, Paoluzzi L, Bertulli R, Hindi N, Montemurro M, Rothermundt C, Cocchi S, Salguero-Aranda C, Donati D, Martin JD, Abdelhamid Ahmed AH, Mazzocca A, Carretta E, Cesari M, Pierini M, Righi A, Sbaraglia M, Laginestra MA, Scotlandi K, Dei Tos AP, Ibrahim T, Stacchiotti S, Vincenzi B
Eur J Cancer 2023 Apr;183:11-23. Epub 2023 Jan 18 doi: 10.1016/j.ejca.2023.01.003. PMID: 36791667
Molecular testing of soft tissue tumors.
Rottmann D, Abdulfatah E, Pantanowitz L
Diagn Cytopathol 2023 Jan;51(1):12-25. Epub 2022 Jul 9 doi: 10.1002/dc.25013. PMID: 35808975Free PMC Article
The 2020 WHO Classification: What's New in Soft Tissue Tumor Pathology?
Kallen ME, Hornick JL
Am J Surg Pathol 2021 Jan;45(1):e1-e23. doi: 10.1097/PAS.0000000000001552. PMID: 32796172
Round Cell Sarcomas: Newcomers and Diagnostic Approaches.
Nagy A, Somers GR
Surg Pathol Clin 2020 Dec;13(4):763-782. Epub 2020 Oct 9 doi: 10.1016/j.path.2020.08.004. PMID: 33183732

Clinical prediction guides

Updates on WHO classification for small round cell tumors: Ewing sarcoma vs. everything else.
Dehner CA, Lazar AJ, Chrisinger JSA
Hum Pathol 2024 May;147:101-113. Epub 2024 Jan 26 doi: 10.1016/j.humpath.2024.01.007. PMID: 38280658
A global collaboRAtive study of CIC-rearranged, BCOR::CCNB3-rearranged and other ultra-rare unclassified undifferentiated small round cell sarcomas (GRACefUl).
Palmerini E, Gambarotti M, Italiano A, Nathenson MJ, Ratan R, Dileo P, Provenzano S, Jones RL, DuBois SG, Martin-Broto J, de Alava E, Baldi GG, Grignani G, Ferraresi V, Brunello A, Paoluzzi L, Bertulli R, Hindi N, Montemurro M, Rothermundt C, Cocchi S, Salguero-Aranda C, Donati D, Martin JD, Abdelhamid Ahmed AH, Mazzocca A, Carretta E, Cesari M, Pierini M, Righi A, Sbaraglia M, Laginestra MA, Scotlandi K, Dei Tos AP, Ibrahim T, Stacchiotti S, Vincenzi B
Eur J Cancer 2023 Apr;183:11-23. Epub 2023 Jan 18 doi: 10.1016/j.ejca.2023.01.003. PMID: 36791667
Molecular classification and outcome of children with rare CNS embryonal tumors: results from St. Jude Children's Research Hospital including the multi-center SJYC07 and SJMB03 clinical trials.
Liu APY, Dhanda SK, Lin T, Sioson E, Vasilyeva A, Gudenas B, Tatevossian RG, Jia S, Neale G, Bowers DC, Hassall T, Partap S, Crawford JR, Chintagumpala M, Bouffet E, McCowage G, Broniscer A, Qaddoumi I, Armstrong G, Wright KD, Upadhyaya SA, Vinitsky A, Tinkle CL, Lucas J, Chiang J, Indelicato DJ, Sanders R, Klimo P Jr, Boop FA, Merchant TE, Ellison DW, Northcott PA, Orr BA, Zhou X, Onar-Thomas A, Gajjar A, Robinson GW
Acta Neuropathol 2022 Oct;144(4):733-746. Epub 2022 Aug 18 doi: 10.1007/s00401-022-02484-7. PMID: 35982322Free PMC Article
Diagnostic Immunohistochemistry for Soft Tissue and Bone Tumors: An Update.
Schaefer IM, Hornick JL
Adv Anat Pathol 2018 Nov;25(6):400-412. doi: 10.1097/PAP.0000000000000204. PMID: 30134255Free PMC Article
Evaluation of ETV4 and WT1 expression in CIC-rearranged sarcomas and histologic mimics.
Hung YP, Fletcher CD, Hornick JL
Mod Pathol 2016 Nov;29(11):1324-1334. Epub 2016 Jul 22 doi: 10.1038/modpathol.2016.140. PMID: 27443513

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