Open Access

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

  • Published online on: February 7, 2022     https://doi.org/10.3892/mco.2022.2510
  • Article Number: 77
  • Copyright: © Suzuki et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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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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April-2022
Volume 16 Issue 4

Print ISSN: 2049-9450
Online ISSN:2049-9469

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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