Clin Pediatr Hematol Oncol.  2022 Oct;29(2):60-64. 10.15264/cpho.2022.29.2.60.

Multimodal Treatment of Sarcomas Linked to BCOR-CCNB3 Fusion in Pediatrics: A 3-Patient Case Series

• Omar S
• Albritton K
• Heym K
• Wang J
• Ray A

• KMID: 2534764
• DOI: http://doi.org/10.15264/cpho.2022.29.2.60

Abstract

In 2012, a new neoplasm was classified according to fusion of the B-cell lymphoma (BCL-6) corepressor (BCOR) gene and the testis-specific cyclin B3 (CCNB3) gene on the X-chromosome, known as a BCOR-CCNB3 fusion positive sarcoma. Traditionally, BCOR sarcomas have been classified as ‘Ewing-like’ due to similarities in morphology. However, BCOR-CCNB3 fusion positive sarcomas are molecularly and genetically distinct. Previous studies have focused on clinical and pathologic characterization of this specific malignancy, but standard treatment modalities are not well documented. We present three pediatric patients diagnosed with BCOR-CCNB3 sarcomas. A twoyear-old girl and a 16-year-old boy were treated using a five-drug therapy consisting of vincristine, doxorubicin, etoposide, ifosfamide, and cyclophosphamide. A 12-yearold girl received a two-drug therapy using a combination of ifosfamide and doxorubicin. All three patients are in remission following chemotherapy and surgery, confirming the effectiveness and safety of the outlined regimens. There is a lack of consensus regarding an appropriate therapy algorithm for Ewing sarcoma patients with BCOR. The present study adds to the extant literature by detailing effective, yet varying treatment modalities.

Keyword

BCOR sarcoma; BCOR-CCNB3 fusion; Chemotherapy; Pediatric oncology