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CIC-DUX Sarcoma

MedGen UID:: 859358
•Concept ID:: C3899764
•: Neoplastic Process

Synonyms:	CIC-Rearranged Sarcoma; CIC-rearranged sarcoma
SNOMED CT:	CIC-rearranged sarcoma (1186889001)

Definition

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

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CIC-DUX Sarcoma

Disorder by Site
- Connective and Soft Tissue Disorder
  - Connective and soft tissue neoplasm
    - Connective tissue neoplasm
      - Small cell sarcoma
        EWSR1-negative small round cell tumor
        CIC-DUX Sarcoma
        CIC-DUX4 Sarcoma
        CIC-DUX4L Sarcoma

Recent clinical studies

Etiology

Clinical characteristics and outcomes for children, adolescents and young adults with "CIC-fused" or "BCOR-rearranged" soft tissue sarcomas: A multi-institutional European retrospective analysis.

Sparber-Sauer M, Corradini N, Affinita MC, Milano GM, Pierron G, Carton M, Tirode F, Pissaloux D, Alaggio R, Vokuhl C, Bisogno G, Berlanga P, Ferrari A, Orbach D
Cancer Med 2023 Jul;12(13):14346-14359. Epub 2023 May 22 doi: 10.1002/cam4.6113. PMID: 37212486 Free PMC Article

Fluorescent In Situ Hybridization for TP53 in the Diagnosis of Pediatric Osteogenic Sarcoma.

Marrano P, Shago M, Somers GR, Thorner PS
Am J Surg Pathol 2018 Jun;42(6):744-749. doi: 10.1097/PAS.0000000000001054. PMID: 29543677

The utility of ETV1, ETV4 and ETV5 RNA in-situ hybridization in the diagnosis of CIC-DUX sarcomas.

Smith SC, Palanisamy N, Martin E, Almenara J, McHugh JB, Choi EK, Lucas DR, Betz BL, Thomas D, Patel RM
Histopathology 2017 Mar;70(4):657-663. Epub 2016 Dec 16 doi: 10.1111/his.13112. PMID: 27790742

CIC-DUX sarcomas demonstrate frequent MYC amplification and ETS-family transcription factor expression.

Smith SC, Buehler D, Choi EY, McHugh JB, Rubin BP, Billings SD, Balzer B, Thomas DG, Lucas DR, Goldblum JR, Patel RM
Mod Pathol 2015 Jan;28(1):57-68. Epub 2014 Jun 20 doi: 10.1038/modpathol.2014.83. PMID: 24947144

See all (4)

Diagnosis

Ewing-Like Sarcoma: A Case of a Primary Cutaneous Primitive Neuroectodermal Tumor With Atypical Immunohistochemistry and Lack of Fusion Oncogene Detection.

Labadie JG, Florek AG, VandenBoom T, Yazdan P, Krunic AL
Am J Dermatopathol 2018 Dec;40(12):890-893. doi: 10.1097/DAD.0000000000001237. PMID: 30067549

Fluorescent In Situ Hybridization for TP53 in the Diagnosis of Pediatric Osteogenic Sarcoma.

Marrano P, Shago M, Somers GR, Thorner PS
Am J Surg Pathol 2018 Jun;42(6):744-749. doi: 10.1097/PAS.0000000000001054. PMID: 29543677

The utility of ETV1, ETV4 and ETV5 RNA in-situ hybridization in the diagnosis of CIC-DUX sarcomas.

Smith SC, Palanisamy N, Martin E, Almenara J, McHugh JB, Choi EK, Lucas DR, Betz BL, Thomas D, Patel RM
Histopathology 2017 Mar;70(4):657-663. Epub 2016 Dec 16 doi: 10.1111/his.13112. PMID: 27790742

Superficial EWSR1-negative undifferentiated small round cell sarcoma with CIC/DUX4 gene fusion: a new variant of Ewing-like tumors with locoregional lymph node metastasis.

Machado I, Cruz J, Lavernia J, Rubio L, Campos J, Barrios M, Grison C, Chene V, Pierron G, Delattre O, Llombart-Bosch A
Virchows Arch 2013 Dec;463(6):837-42. Epub 2013 Nov 10 doi: 10.1007/s00428-013-1499-9. PMID: 24213312

See all (4)

Prognosis

Fluorescent In Situ Hybridization for TP53 in the Diagnosis of Pediatric Osteogenic Sarcoma.

Marrano P, Shago M, Somers GR, Thorner PS
Am J Surg Pathol 2018 Jun;42(6):744-749. doi: 10.1097/PAS.0000000000001054. PMID: 29543677

See all (1)

Clinical prediction guides

Fluorescent In Situ Hybridization for TP53 in the Diagnosis of Pediatric Osteogenic Sarcoma.

Marrano P, Shago M, Somers GR, Thorner PS
Am J Surg Pathol 2018 Jun;42(6):744-749. doi: 10.1097/PAS.0000000000001054. PMID: 29543677

The utility of ETV1, ETV4 and ETV5 RNA in-situ hybridization in the diagnosis of CIC-DUX sarcomas.

Smith SC, Palanisamy N, Martin E, Almenara J, McHugh JB, Choi EK, Lucas DR, Betz BL, Thomas D, Patel RM
Histopathology 2017 Mar;70(4):657-663. Epub 2016 Dec 16 doi: 10.1111/his.13112. PMID: 27790742

CIC-DUX sarcomas demonstrate frequent MYC amplification and ETS-family transcription factor expression.

See all (3)

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CIC-DUX Sarcoma

Definition

Term Hierarchy

Recent clinical studies

Etiology

Diagnosis

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

Related information

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