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.
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BCOR-CCNB3 fusion-positive sarcoma (BCS) is a recently recognized subtype of undifferentiated round-cell sarcoma that occurs predominantly in children and adolescents. Owing to its rarity, an optimal treatment strategy has not been fully established, and management has historically followed treatment protocols for Ewing sarcoma. Although radiotherapy is a potential treatment option, the role of carbon-ion radiotherapy (CIRT) in treating this tumor has not been previously reported. A 14-year-old boy presented with posterior neck pain. Magnetic resonance imaging demonstrated a tumor arising from the posterior elements of the third cervical vertebra. Needle biopsy revealed a round-cell sarcoma without EWS gene rearrangement, and the patient was initially treated for Ewing-like sarcoma. Systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide was initiated, resulting in tumor shrinkage. Subsequently, the patient underwent CIRT (70.4 Gy in 16 fractions), followed by adjuvant chemotherapy. Magnetic resonance imaging after treatment completion showed no evidence of residual tumor. More than 12 years after treatment, the patient has remained asymptomatic without neurological deficits, tumor recurrence, or distant metastasis, although cervical kyphosis was radiographically observed. Subsequent genetic analysis confirmed the presence of the BCOR-CCNB3 fusion gene. To the best of our knowledge, this is the first reported case of BCS treated with CIRT. This case demonstrates that CIRT can achieve durable local control and suggests that it may represent a promising treatment option for BCS when surgical resection is challenging.