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BCOR-CCNB3 sarcoma

MedGen UID:
1791499
•Concept ID:
C5555058
•
Neoplastic Process
Synonyms: BCOR-CCNB3 Fusion-Positive Sarcoma; BCOR-CCNB3 Sarcoma; BCOR-Rearranged Sarcoma; BCOR::CCNB3 Fusion-Positive Sarcoma; BCOR::CCNB3 Sarcoma
 
Monarch Initiative: MONDO:0958296

Definition

A group of rare primitive round cell sarcomas characterized by the presence of BCOR-CCNB3 fusion gene. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBCOR-CCNB3 sarcoma

Professional guidelines

PubMed

Strategies for the Treatment of Infantile Soft Tissue Sarcomas With BCOR Alterations.
Merjaneh N, Kim H, Escoto H, Metts J, Ray A, Bukowinski A, LeBlanc Z, Fair D, Watanbe M, Alva E, Todd K, Daley J, Hartt D, Cramer SL, Szabo S, Pressey JG
J Pediatr Hematol Oncol 2023 Aug 1;45(6):315-321. Epub 2023 Jan 10 doi: 10.1097/MPH.0000000000002620. PMID: 36706311Free PMC Article
Immunophenotype-Genotype Correlations in Clear Cell Sarcoma of Kidney-An Evaluation of Diagnostic Ancillary Studies.
Kenny C, Grehan D, Ulas M, Banga GB, Coulomb A, Vokuhl C, O'Sullivan MJ
Pediatr Dev Pathol 2020 Sep-Oct;23(5):345-351. Epub 2020 May 4 doi: 10.1177/1093526620910658. PMID: 32364435
Defining Ewing and Ewing-like small round cell tumors (SRCT): The need for molecular techniques in their categorization and differential diagnosis. A study of 200 cases.
Machado I, Navarro L, Pellin A, Navarro S, Agaimy A, Tardío JC, Karseladze A, Petrov S, Scotlandi K, Picci P, Llombart-Bosch A
Ann Diagn Pathol 2016 Jun;22:25-32. Epub 2016 Mar 14 doi: 10.1016/j.anndiagpath.2016.03.002. PMID: 27180056

Recent clinical studies

Etiology

Rare Case of BCOR::CCNB3 Sarcoma of Bone.
Arai Y, Yazawa Y, Torigoe T, Imanishi J, Motoi T, Yasuda M, Tanaka R, Fukushima T, Watanabe A, Ohta A, Shimizu Y, Kadono Y, Saita K
Am J Case Rep 2023 Mar 14;24:e938158. doi: 10.12659/AJCR.938158. PMID: 36915189Free PMC Article
Clinicopathologic and molecular analysis of a BCOR-CCNB3 undifferentiated sarcoma of the kidney reveals significant epigenetic alterations.
Hagoel TJ, Cortes Gomez E, Gupta A, Twist CJ, Kozielski R, Martin JC, Gao L, Kuechle J, Singh PK, Lynch M, Wei L, Liu S, Wang J, Ohm JE
Cold Spring Harb Mol Case Stud 2022 Jan;8(1) Epub 2022 Jan 10 doi: 10.1101/mcs.a005942. PMID: 34819304Free PMC Article
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Congenital undifferentiated sarcoma associated to BCOR-CCNB3 gene fusion.
Alfaro-Cervello C, Andrade-Gamarra V, Nieto G, Navarro L, Martín-Vañó S, García de la Torre JP, Bengoa Caamaño M, García Mauriño ML, Noguera R, Navarro S
Pathol Res Pract 2017 Nov;213(11):1435-1439. Epub 2017 Jul 14 doi: 10.1016/j.prp.2017.07.012. PMID: 28756981
Screening of BCOR-CCNB3 sarcoma using immunohistochemistry for CCNB3: A clinicopathological report of three pediatric cases.
Shibayama T, Okamoto T, Nakashima Y, Kato T, Sakurai T, Minamiguchi S, Kataoka TR, Shibuya S, Yoshizawa A, Toguchida J, Haga H
Pathol Int 2015 Aug;65(8):410-4. Epub 2015 Jun 3 doi: 10.1111/pin.12319. PMID: 26037154

Diagnosis

Rare Case of BCOR::CCNB3 Sarcoma of Bone.
Arai Y, Yazawa Y, Torigoe T, Imanishi J, Motoi T, Yasuda M, Tanaka R, Fukushima T, Watanabe A, Ohta A, Shimizu Y, Kadono Y, Saita K
Am J Case Rep 2023 Mar 14;24:e938158. doi: 10.12659/AJCR.938158. PMID: 36915189Free PMC Article
BCOR-CCNB3 sarcoma arising in the pharynx.
Eguchi K, Omura G, Shimoi T, Kageyama D, Igaki H, Abe Y, Watanabe T, Aihara Y, Sakai A, Matsumoto Y, Sakai T, Yonemori K, Mori T, Yoshida A, Yoshimoto S
Auris Nasus Larynx 2023 Aug;50(4):618-622. Epub 2022 May 8 doi: 10.1016/j.anl.2022.04.012. PMID: 35545465
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Screening of BCOR-CCNB3 sarcoma using immunohistochemistry for CCNB3: A clinicopathological report of three pediatric cases.
Shibayama T, Okamoto T, Nakashima Y, Kato T, Sakurai T, Minamiguchi S, Kataoka TR, Shibuya S, Yoshizawa A, Toguchida J, Haga H
Pathol Int 2015 Aug;65(8):410-4. Epub 2015 Jun 3 doi: 10.1111/pin.12319. PMID: 26037154
BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.
Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP, Kindblom LG
Am J Surg Pathol 2014 Oct;38(10):1307-18. doi: 10.1097/PAS.0000000000000223. PMID: 24805859

Prognosis

BCOR-CCNB3 sarcoma arising in the pharynx.
Eguchi K, Omura G, Shimoi T, Kageyama D, Igaki H, Abe Y, Watanabe T, Aihara Y, Sakai A, Matsumoto Y, Sakai T, Yonemori K, Mori T, Yoshida A, Yoshimoto S
Auris Nasus Larynx 2023 Aug;50(4):618-622. Epub 2022 May 8 doi: 10.1016/j.anl.2022.04.012. PMID: 35545465
Detection of BCOR gene rearrangement in Ewing-like sarcoma: an important diagnostic tool.
Li L, Zhang M, Chen S, Sun X, Xu H, Li L, Zhang T, Huang X, Ye H, Ding Y
Diagn Pathol 2021 Jun 8;16(1):50. doi: 10.1186/s13000-021-01114-2. PMID: 34103053Free PMC Article
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Clinicopathologic Diversity of Undifferentiated Sarcoma With BCOR-CCNB3 Fusion: Analysis of 11 Cases With a Reappraisal of the Utility of Immunohistochemistry for BCOR and CCNB3.
Matsuyama A, Shiba E, Umekita Y, Nosaka K, Kamio T, Yanai H, Miyasaka C, Watanabe R, Ito I, Tamaki T, Hayashi S, Hisaoka M
Am J Surg Pathol 2017 Dec;41(12):1713-1721. doi: 10.1097/PAS.0000000000000934. PMID: 28877060
BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.
Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP, Kindblom LG
Am J Surg Pathol 2014 Oct;38(10):1307-18. doi: 10.1097/PAS.0000000000000223. PMID: 24805859

Clinical prediction guides

Detection of BCOR gene rearrangement in Ewing-like sarcoma: an important diagnostic tool.
Li L, Zhang M, Chen S, Sun X, Xu H, Li L, Zhang T, Huang X, Ye H, Ding Y
Diagn Pathol 2021 Jun 8;16(1):50. doi: 10.1186/s13000-021-01114-2. PMID: 34103053Free PMC Article
Fine-Needle Aspiration Features of BCOR-CCNB3 Sarcoma.
Gajdzis P, Laé M, Pierron G, Brisse HJ, Orbach D, Fréneaux P, Laurence V, Klijanienko J
Am J Clin Pathol 2020 Feb 8;153(3):315-321. doi: 10.1093/ajcp/aqz159. PMID: 31679010
BCOR-CCNB3 Fusion Positive Sarcomas: A Clinicopathologic and Molecular Analysis of 36 Cases With Comparison to Morphologic Spectrum and Clinical Behavior of Other Round Cell Sarcomas.
Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, Fletcher CDM, Antonescu CR
Am J Surg Pathol 2018 May;42(5):604-615. doi: 10.1097/PAS.0000000000000965. PMID: 29300189Free PMC Article
Screening of BCOR-CCNB3 sarcoma using immunohistochemistry for CCNB3: A clinicopathological report of three pediatric cases.
Shibayama T, Okamoto T, Nakashima Y, Kato T, Sakurai T, Minamiguchi S, Kataoka TR, Shibuya S, Yoshizawa A, Toguchida J, Haga H
Pathol Int 2015 Aug;65(8):410-4. Epub 2015 Jun 3 doi: 10.1111/pin.12319. PMID: 26037154
BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.
Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP, Kindblom LG
Am J Surg Pathol 2014 Oct;38(10):1307-18. doi: 10.1097/PAS.0000000000000223. PMID: 24805859

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