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BCOR‑CCNB3 sarcoma arising in the proximal tibia: A case report

  • Authors:
    • Kayo Suzuki
    • Taketoshi Yasuda
    • Yuki Haruhara
    • Kenta Watanabe
    • Keiko Nomura
    • Masahiko Kanamori
    • Yoshiharu Kawaguchi
  • View Affiliations / Copyright

    Affiliations: Department of Orthopedic Surgery, Faculty of Medicine, University of Toyama, Toyama 930‑0194, Japan, Department of Pediatrics, University of Toyama, Toyama 930‑0194, Japan, Department of Human Science 1, University of Toyama, Toyama 930‑0194, Japan
    Copyright: © Suzuki et al. This is an open access article distributed under the terms of Creative Commons Attribution License.
  • Article Number: 77
    |
    Published online on: February 7, 2022
       https://doi.org/10.3892/mco.2022.2510
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Abstract

BCL6 corepressor-cyclin B3 (BCOR‑CCNB3) fusion sarcoma was classified as an emerging subgroup of undifferentiated small round cell sarcoma in 2020. The incidence of BCOR‑CCNB3 fusion sarcoma is reportedly 1.5‑14% among undifferentiated unclassified sarcomas, representing a rare entity among primary malignant bone tumors. The present study reports a case of BCOR‑CCNB3 fusion sarcoma in the proximal tibia of a boy. A 12‑year‑old boy presented with a 6‑month history of knee pain and a slowly growing mass in the anteromedial aspect of the left proximal tibia. Plain radiography and computed tomography of the knee demonstrated a lytic lesion with cortical destruction of the proximal tibia. Magnetic resonance imaging showed the bone tumor expanding into soft tissue with almost homogeneous hypointensity on T1‑weighted imaging and slightly hyperintensity on T2‑weighted imaging. On histopathological evaluation, the tumor comprised a proliferation of small, round to ovoid‑shaped mesenchymal cells without osteoid formation. Histopathologically, BCOR‑CCNB3 sarcoma of bone was finally diagnosed based on immunohistochemical staining and additional molecular analyses. The patient underwent bone tumor resection followed by pre‑ and post‑operative chemotherapy according to a Ewing sarcoma protocol. The patient showed no evidence of local recurrence or distant metastasis at 12 months after completion of adjuvant chemotherapy. We present herein an additional case of BCOR‑CCNB3 sarcoma of the proximal tibia, and review the relevant literature on BCOR‑CCNB3 sarcoma of bone.
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1 

Bridge JA: Undifferentiated small round cell sarcomas of bone and soft tissue. In: WHO classification of tumours of soft tissue and bone tumours. IARC Press, Lyon, pp326-335, 2020.

2 

Turc-Carel C, Aurias A, Mugneret F, Lizard S, Sidaner I, Volk C, Thiery JP, Olschwang S, Philip I and Berger MP: Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12). Cancer Genet Cytogenet. 32:229–238. 1988.PubMed/NCBI View Article : Google Scholar

3 

Kawamura-Saito M, Yamazaki Y, Kaneko K, Kawaguchi N, Kanda H, Mukai H, Gotoh T, Motoi T, Fukayama M, Aburatani H, et al: Fusion between CIC and DUX4 up-regulates PEA3 family genes in Ewing-like sarcomas with t(4;19)(q35;q13) translocation. Hum Mol Genet. 15:2125–2137. 2006.PubMed/NCBI View Article : Google Scholar

4 

Pierron G, Tirode F, Lucchesi C, Reynaud S, Ballet S, Cohen-Gogo S, Perrin V, Coindre JM and Delattre O: A new subtype of bone sarcoma defined by BCOR-CCNB3 gene fusion. Nat Genet. 44:461–466. 2012.PubMed/NCBI View Article : Google Scholar

5 

Sbaraglia M, Righi A, Gambarotti M and Dei Tos AP: Ewing sarcoma and Ewing-like tumors. Virchows Arch. 476:109–119. 2020.PubMed/NCBI View Article : Google Scholar

6 

Antonescu C: Round cell sarcomas beyond Ewing: Emerging entities. Histopathology. 64:26–37. 2014.PubMed/NCBI View Article : Google Scholar

7 

Le Loarer F, Pissaloux D, Coindre JM, Tirode F and Vince DR: Update on families of round cell sarcomas other than classical Ewing sarcomas. Surg Pathol Clin. 10:587–620. 2017.PubMed/NCBI View Article : Google Scholar

8 

Specht K, Zhang L, Sung YS, Nucci M, Dry S, Vaiyapuri S, Richter GH, Fletcher CD and Antonescu CR: Novel BCOR-MAML3 and ZC3H7B-BCOR gene fusions in undifferentiated small blue round cell sarcomas. Am J Surg Pathol. 40:433–442. 2016.PubMed/NCBI View Article : Google Scholar

9 

Kao YC, Sung YS, Zhang L, Huang SC, Argani P, Chung CT, Graf NS, Wright DC, Kellie SJ, Agaram NP, et al: Recurrent BCOR internal tandem duplication and YWHAE-NUTM2B fusions in soft tissue undifferentiated round cell sarcoma of infancy: Overlapping genetic features with clear cell sarcoma of kidney. Am J Surg Pathol. 40:1009–1020. 2016.PubMed/NCBI View Article : Google Scholar

10 

Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, et al: Addition of ifosfamide and etoposide to standard chemotherapy for Ewing's sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med. 348:694–701. 2003.PubMed/NCBI View Article : Google Scholar

11 

Yoshida Y, Osaka S and Ryu J: Reconstruction of the knee extensor mechanism in patients with a malignant bone tumor of the proximal tibia. Surg Today. 40:646–649. 2010.PubMed/NCBI View Article : Google Scholar

12 

Cohen-Gogo S, Cellier C, Coindre JM, Mosseri V, Pierron G, Guillemet C, Italiano A, Brugières L, Orbach D, Laurence V, 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. 61:2191–2198. 2014.PubMed/NCBI View Article : Google Scholar

13 

Puls F, Niblett A, Marland G, Gaston CL, Douis H, Mangham DC, Sumathi VP and Kindblom LG: BCOR-CCNB3 (Ewing-like) sarcoma: A clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma. Am J Surg Pathol. 38:1307–1318. 2014.PubMed/NCBI View Article : Google Scholar

14 

Peters TL, Kumar V, Polikepahad S, Lin FY, Sarabia SF, Liang Y, Wang WL, Lazar AJ, Doddapaneni H, Chao H, et al: BCOR-CCNB3 fusions are frequent in undifferentiated sarcomas of male children. Mod Pathol. 28:575–586. 2015.PubMed/NCBI View Article : Google Scholar

15 

Shibayama T, Okamoto T, Nakashima Y, Kato T, Sakurai T, Minamiguchi S, Kataoka TR, Shibuya S, Yoshizawa A, Toguchida J and Haga H: Screening of BCOR-CCNB3 sarcoma using immunohistochemistry for CCNB3: A clinicopathological report of three pediatric cases. Pathol Int. 65:410–414. 2015.PubMed/NCBI View Article : Google Scholar

16 

Ludwig K, Alaggio R, Zin A, Peron M, Guzzardo V, Benini S, Righi A and Gambarotti M: BCOR-CCNB3 undifferentiated sarcoma-does immunohistochemistry help in the identification? Pediatr Dev Pathol. 20:321–329. 2017.PubMed/NCBI View Article : Google Scholar

17 

Yamada Y, Kuda M, Kohashi K, Yamamoto H, Takemoto J, Ishii T, Iura K, Maekawa A, Bekki H, Ito T, et al: Histological and immunohistochemical characteristics of undifferentiated small round cell sarcomas associated with CIC-DUX4 and BCOR-CCNB3 fusion genes. Virchows Arch. 470:373–380. 2017.PubMed/NCBI View Article : Google Scholar

18 

Matsuyama A, Shiba E, Umekita Y, Nosaka K, Kamio T, Yanai H, Miyasaka C, Watanabe R, Ito I, Tamaki T, 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. 41:1713–1721. 2017.PubMed/NCBI View Article : Google Scholar

19 

Krskova L, Kabickova E, Drahokoupilova E, Kopeckova K, Plank L, Vitkova P, Mrhalova M, Zamecnik J and Kodet R: An undifferentiated sarcoma with BCOR-CCNB3 fusion transcript-pathological and clinical retrospective study. Neoplasma. 65:630–636. 2018.PubMed/NCBI View Article : Google Scholar

20 

Kao YC, Owosho AA, Sung YS, Zhang L, Fujisawa Y, Lee JC, Wexler L, Argani P, Swanson D, Dickson BC, 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. 42:604–615. 2018.PubMed/NCBI View Article : Google Scholar

21 

Rekhi B, Kembhavi P, Mishra SN, Shetty O, Bajpai J and 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. 150:557–574. 2019.PubMed/NCBI View Article : Google Scholar

22 

Brady EJ, Hameed M, Tap WD and Hwang S: Imaging features and clinical course of undifferentiated round cell sarcomas with CIC-DUX4 and BCOR-CCNB3 translocations. Skeletal Radiol. 50:521–529. 2021.PubMed/NCBI View Article : Google Scholar

23 

Khoury JD: Ewing sarcoma family of tumors. Adv Anat Pathol. 12:212–220. 2005.PubMed/NCBI View Article : Google Scholar

24 

Mar WA, Taljanovic MS, Bagatell R, Graham AR, Speer DP, Hunter TB and Rogers LF: Update on imaging and treatment of Ewing sarcoma family tumors: What the radiologist needs to know. J Comput Assist Tomogr. 32:108–118. 2008.PubMed/NCBI View Article : Google Scholar

25 

Sirisena UDN, Rajakulasingam R and Saifuddin A: Imaging of bone and soft tissue BCOR-rearranged sarcoma. Skeletal Radiol. 50:1291–1301. 2021.PubMed/NCBI View Article : Google Scholar

26 

Murphey MD, Senchak LT, Mambalam PK, Logie CI, Klassen-Fischer MK and Kransdorf MJ: From the radiologic pathology archives: Ewing sarcoma family of tumors: Radiologic-pathologic correlation. Radiographics. 33:803–831. 2013.PubMed/NCBI View Article : Google Scholar

27 

Limb D, Dreghorn C, Murphy JK and Mannion R: Primary lymphoma of bone. Int Orthop. 18:180–183. 1994.PubMed/NCBI View Article : Google Scholar

28 

Akahane T, Shimizu T, Isobe K, Yoshimura Y and Kato H: Serum soluble interleukin-2 receptor levels in patients with malignant lymphoma of bone. J Orthop Sci. 14:248–252. 2009.PubMed/NCBI View Article : Google Scholar

29 

Yarmish G, Klein MJ, Landa J, Lefkowitz RA and Hwang S: Imaging characteristics of primary osteosarcoma: Nonconventional subtypes. Radiographics. 30:1653–1672. 2010.PubMed/NCBI View Article : Google Scholar

30 

Zhong J, Hu Y, Si L, Geng J, Xing Y, Jiao Q, Zhang H and Yao W: Clarifying prognostic factors of small cell osteosarcoma: A pooled analysis of 20 cases and the literature. J Bone Oncol. 24(100305)2020.PubMed/NCBI View Article : Google Scholar

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Copy and paste a formatted citation
Spandidos Publications style
Suzuki K, Yasuda T, Haruhara Y, Watanabe K, Nomura K, Kanamori M and Kawaguchi Y: <em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report. Mol Clin Oncol 16: 77, 2022.
APA
Suzuki, K., Yasuda, T., Haruhara, Y., Watanabe, K., Nomura, K., Kanamori, M., & Kawaguchi, Y. (2022). <em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report. Molecular and Clinical Oncology, 16, 77. https://doi.org/10.3892/mco.2022.2510
MLA
Suzuki, K., Yasuda, T., Haruhara, Y., Watanabe, K., Nomura, K., Kanamori, M., Kawaguchi, Y."<em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report". Molecular and Clinical Oncology 16.4 (2022): 77.
Chicago
Suzuki, K., Yasuda, T., Haruhara, Y., Watanabe, K., Nomura, K., Kanamori, M., Kawaguchi, Y."<em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report". Molecular and Clinical Oncology 16, no. 4 (2022): 77. https://doi.org/10.3892/mco.2022.2510
Copy and paste a formatted citation
x
Spandidos Publications style
Suzuki K, Yasuda T, Haruhara Y, Watanabe K, Nomura K, Kanamori M and Kawaguchi Y: <em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report. Mol Clin Oncol 16: 77, 2022.
APA
Suzuki, K., Yasuda, T., Haruhara, Y., Watanabe, K., Nomura, K., Kanamori, M., & Kawaguchi, Y. (2022). <em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report. Molecular and Clinical Oncology, 16, 77. https://doi.org/10.3892/mco.2022.2510
MLA
Suzuki, K., Yasuda, T., Haruhara, Y., Watanabe, K., Nomura, K., Kanamori, M., Kawaguchi, Y."<em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report". Molecular and Clinical Oncology 16.4 (2022): 77.
Chicago
Suzuki, K., Yasuda, T., Haruhara, Y., Watanabe, K., Nomura, K., Kanamori, M., Kawaguchi, Y."<em>BCOR‑CCNB3</em> sarcoma arising in the proximal tibia: A case report". Molecular and Clinical Oncology 16, no. 4 (2022): 77. https://doi.org/10.3892/mco.2022.2510
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