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Imaging of bone and soft tissue BCOR-rearranged sarcoma

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Abstract

With recent advances in molecular research, an ever-increasing number of undifferentiated round cell sarcomas without the characteristic gene fusions of Ewing sarcoma are being discovered. One specific subtype termed BCOR-rearranged sarcoma belongs to this group. Previously termed ‘Ewing-like’ sarcoma, it was formally included with undifferentiated round cell tumours in the 2013 WHO Classification of Soft Tissue and Bone Tumours. However, in the 2020 WHO Classification, BCOR-sarcoma is now recognized as a distinct entity due to particular morphological and immunohistochemical features and differing clinical outcomes. As with classical Ewing sarcoma, osseous BCOR-rearranged sarcoma is an aggressive tumour with a similar clinical presentation. However, there are only a small handful of case series and isolated reports detailing the imaging characteristics, typically demonstrating an aggressive bone lesion with a large soft tissue mass. Soft tissue BCOR-sarcoma is even rarer. The aim of the current review is to describe the patient demographics, lesion locations and various imaging characteristics of histologically proven cases of musculoskeletal bone and soft tissue BCOR-sarcoma as described in the literature.

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References

  1. Arndt CA, Rose PS, Folpe AL, Laack NN. Common musculoskeletal tumors of childhood and adolescence. Mayo Clin Proc. 2012;87(5):475–87.

    Article  Google Scholar 

  2. Lessnick SL, Dei Tos AP, Sorensen PH, et al. Small round cell sarcomas. Semin Oncol. 2009;36(4):338–46.

    Article  CAS  Google Scholar 

  3. Fletcher CDM, Bridge JA, Hogendoorn PCW, et al. WHO classification of tumors of soft tissue and bone. 4th ed. Lyon: IARC; 2013.

    Google Scholar 

  4. Antonescu CR, Bridge JA, Cunha IW, et al. WHO classification of soft tissue and bone tumors. 5th ed. Lyon: IARC; 2020.

    Google Scholar 

  5. Renzi S, Anderson ND, Light N, Gupta A. Ewing-like sarcoma: an emerging family of round cell sarcomas. J Cell Physiol. 2019;234(6):7999–8007.

    Article  CAS  Google Scholar 

  6. Pierron G, Tirode F, Lucchesi C, et al. A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion. Nat Genet. 2012;44(4):461–6.

    Article  CAS  Google Scholar 

  7. Nguyen TB, Manova K, Capodieci P, et al. Characterization and expression of mammalian cyclin b3, a prepachytene meiotic cyclin. J Biol Chem. 2002;277(44):41960–9.

    Article  CAS  Google Scholar 

  8. Specht K, Zhang L, Sung YS, et al. Novel BCOR-MAML3 and ZC3H7B-BCOR gene fusions in undifferentiated small blue round cell sarcomas. Am J Surg Pathol. 2016;40:433–42.

    Article  Google Scholar 

  9. Cohen-Gogo S, Cellier C, Coindre JM, et al. Ewing-like sarcomas with BCOR-CCNB3 fusion transcript: a clinical, radiological and pathological retrospective study from the Société Française des Cancers de L’Enfant. Pediatr Blood Cancer. 2014;61(12):2191–8.

    Article  Google Scholar 

  10. Puls F, Niblett A, Marland G, et al. BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma. Am J Surg Pathol. 2014;38(10):1307–18.

    Article  Google Scholar 

  11. Peters TL, Kumar V, Polikepahad S, et al. BCOR-CCNB3 fusions are frequent in undifferentiated sarcomas of male children. Mod Pathol. 2015;28(4):575–86.

    Article  CAS  Google Scholar 

  12. Shibayama T, Okamoto T, Nakashima Y, et al. Screening of BCOR-CCNB3 sarcoma using immunohistochemistry for CCNB3: a clinicopathological report of three pediatric cases. Pathol Int. 2015;65(8):410–4.

    Article  Google Scholar 

  13. Ludwig K, Alaggio R, Zin A, et al. BCOR-CCNB3 undifferentiated sarcoma-does immunohistochemistry help in the identification? Pediatr Dev Pathol. 2017;20(4):321–9.

    Article  Google Scholar 

  14. Yamada Y, Kuda M, Kohashi K, et al. Histological and immunohistochemical characteristics of undifferentiated small round cell sarcomas associated with CIC-DUX4 and BCOR-CCNB3 fusion genes. Virchows Arch. 2017;470(4):373–80.

    Article  CAS  Google Scholar 

  15. Matsuyama A, Shiba E, Umekita Y, et al. 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. Am J Surg Pathol. 2017;41(12):1713–21.

    Article  Google Scholar 

  16. Kao YC, Owosho AA, Sung YS, et al. 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. Am J Surg Pathol. 2018;42(5):604–15.

    Article  Google Scholar 

  17. Machado I, Yoshida A, Morales MGN, et al. Review with novel markers facilitates precise categorization of 41 cases of diagnostically challenging, “undifferentiated small round cell tumors”. A clinicopathologic, immunophenotypic and molecular analysis. Ann Diagn Pathol. 2018;34:1–12.

    Article  Google Scholar 

  18. Bharaucha P, Harvilla N, White R, et al. Unusual distal tibia BCOR Sarcoma: a case report and review of imaging features. OAJ. Case Rep. 2019;6–11

  19. Rekhi B, Kembhavi P, Mishra SN, Shetty O, Bajpai J, Puri A. Clinicopathologic features of undifferentiated round cell sarcomas of bone & soft tissues: an attempt to unravel the BCOR-CCNB3- & CIC-DUX4-positive sarcomas. Indian J Med Res. 2019;150(6):557–74.

    Article  CAS  Google Scholar 

  20. Brady EJ, Hameed M, Tap WD, et al. Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skelet Radiol. 2020.

  21. Li WS, Liao IC, Wen MC, Lan HH, Yu SC, Huang HY. BCOR-CCNB3-positive soft tissue sarcoma with round-cell and spindle-cell histology: a series of four cases highlighting the pitfall of mimicking poorly differentiated synovial sarcoma. Histopathology. 2016;69(5):792–801.

    Article  Google Scholar 

  22. Yang Y, Shi H, Zheng J, et al. BCOR-CCNB3 fusion and BCOR internal tandem duplication in undifferentiated round cell sarcoma: a pathologic and molecular study of 5 cases. Am J Transl Res. 2019;11(9):5836–46.

    CAS  PubMed  PubMed Central  Google Scholar 

  23. ESMO Guidelines Working Group, Saeter G. Ewing’s sarcoma of bone: ESMO clinical recommendations for diagnosis, treatment and follow-up [published correction appears in Ann Oncol. 2008 May;19(5):1027–9]. Ann Oncol. 2007;18(Suppl 2):ii79–80.

    Google Scholar 

  24. Caracciolo JT, Temple HT, Letson GD, et al. A modified Lodwick-Madewell grading system for the evaluation of lytic bone lesions. AJR Am J Roentgenol. 2016;207(1):150–6.

    Article  Google Scholar 

  25. Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK, Kransdorf MJ. From the radiologic pathology archives: Ewing sarcoma family of tumors: radiologic-pathologic correlation. Radiographics. 2013;33(3):803–31.

    Article  Google Scholar 

  26. Javery O, Krajewski K, O’Regan K, et al. A to Z of extraskeletal Ewing sarcoma family of tumors in adults: imaging features of primary disease, metastatic patterns, and treatment responses. AJR Am J Roentgenol. 2011;197(6):W1015–22.

    Article  Google Scholar 

  27. Tao HT, Hu Y, Wang JL, et al. Extraskeletal Ewing sarcomas in late adolescence and adults: a study of 37 patients. Asian Pac J Cancer Prev. 2013;14(5):2967–71.

    Article  Google Scholar 

  28. Sbaraglia M, Righi A, Gambarotti M, et al. Ewing sarcoma and Ewing-like tumors. Virchows Arch. 2020;476(1):109–19.

    Article  CAS  Google Scholar 

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Acknowledgements

The authors would like to acknowledge Dr. Roberto Tirabosco (Consultant Histopathologist at the Royal National Orthopaedic Hospital, London) for the histology images.

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Authors and Affiliations

  1. Watford General Hospital, Vicarage Road, Watford, WD18 0HB, UK

    Udawattage Darshana Nadeeka Sirisena

  2. Department of Radiology, Royal National Orthopaedic Hospital, Brockley Hill, Stanmore, Middlesex, London, HA7 4LP, UK

    Ramanan Rajakulasingam & Asif Saifuddin

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  1. Udawattage Darshana Nadeeka Sirisena
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  2. Ramanan Rajakulasingam
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Correspondence to Ramanan Rajakulasingam.

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Sirisena, U.D.N., Rajakulasingam, R. & Saifuddin, A. Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol 50, 1291–1301 (2021). https://doi.org/10.1007/s00256-020-03683-7

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  • DOI: https://doi.org/10.1007/s00256-020-03683-7

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