BCOR-CCNB3 fusion–positive sarcoma treated with chemotherapy and carbon-ion radiotherapy: a case report of long-term disease control of a cervical spine case with a 12-year follow-up - Scorecard - MDSpire
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Management of BCOR-CCNB3 Fusion-Positive Sarcoma with Chemotherapy and Carbon-Ion Radiotherapy: A Case Study Highlighting Long-Term Disease Control in a Cervical Spine Patient Over 12 Years

  • By

  • Naoki Oike

  • Reiko Imai

  • Akira Ogose

  • Takashi Ariizumi

  • Yudai Murayama

  • Tomohiro Miyazaki

  • Kazunaga Kimura

  • Hajime Umezu

  • Hiroyuki Kawashima

  • June 10, 2026

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Clinical Scorecard: Management of BCOR-CCNB3 Fusion-Positive Sarcoma with Chemotherapy and Carbon-Ion Radiotherapy: A Case Study Highlighting Long-Term Disease Control in a Cervical Spine Patient Over 12 Years

At a Glance

CategoryDetail
ConditionBCOR–CCNB3 fusion-positive sarcoma (BCS)
Key MechanismsCharacterized by BCOR–CCNB3 gene fusion resulting from a paracentric inversion on chromosome X.
Target PopulationPredominantly children and adolescents.
Care SettingMultimodal approach including chemotherapy and radiotherapy.

Key Highlights

  • Initial treatment followed protocols for Ewing sarcoma.
  • Systemic chemotherapy resulted in tumor shrinkage.
  • Carbon-ion radiotherapy (CIRT) achieved durable local control.
  • Patient remained asymptomatic without neurological deficits over 12 years.
  • Cervical kyphosis observed radiographically post-treatment.

Guideline-Based Recommendations

Diagnosis

  • Needle biopsy and genetic analysis confirm BCOR–CCNB3 fusion.

Management

  • Systemic chemotherapy with vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide.
  • CIRT as a treatment option for unresectable tumors.

Monitoring & Follow-up

  • Regular MRI assessments to evaluate tumor response.

Risks

  • Potential for spinal cord compression due to tumor location.

Patient & Prescribing Data

14-year-old boy with cervical spine tumor.

Combination of chemotherapy and CIRT resulted in complete response.

Clinical Best Practices

  • Consider CIRT for challenging cases of BCS.
  • Follow established protocols for Ewing-like sarcomas when treating BCS.

Related Resources & Content

Original Source(s)

BCOR-CCNB3 fusion–positive sarcoma treated with chemotherapy and carbon-ion radiotherapy: a case report of long-term disease control of a cervical spine case with a 12-year follow-up

  • by Naoki Oike, Reiko Imai, Akira Ogose, Takashi Ariizumi, Yudai Murayama, Tomohiro Miyazaki, Kazunaga Kimura, Hajime Umezu, Hiroyuki Kawashima

  • June 10, 2026

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