EC Dental Science

BCOR rearranged sarcoma is constituted of an undifferentiated round cell sarcoma characteristically displaying BCOR::CCNB3 genetic rearrangement. Previously designated as Ewing-like sarcoma, tumefaction exhibits nuclear immune reactivity to CCNB3 and BCOR along with co expression of TLE1 and SATB2 genes.

Additionally designated as BCOR::CCNB3 positive sarcoma or undifferentiated round cell sarcoma with BCOR::CCNB3 fusion, BCOR rearranged sarcoma configures ~4% of undifferentiated round cell sarcomas. BCOR rearranged sarcoma is predominantly associated with distant metastasis into pulmonary parenchyma.

BCOR rearranged sarcoma commonly arises within paediatric subjects or young adults with mean age of disease emergence at ~15 years. A male predominance is observed with male to female proportion of ~3:1.

BCOR rearranged sarcoma commonly incriminates bones followed in frequency by soft tissues and diverse viscera as renal parenchyma. Generally, tumefaction is engendered within long bones followed by pelvis and vertebral column [1,2].

1. Whittle SB., et al. “Soft Tissue and Visceral Organ Sarcomas with BCOR Alterations”. Journal of Pediatric Hematology/Oncology 5 (2022): 195-200.
2. Ji T., et al. “Clinical Characteristics of Children with Nasal BCOR-Rearranged Ewing-like Sarcoma: A Case Report and Literature Review”. Ear, Nose and Throat Journal (2022): 1455613221132392.
3. Bouchoucha Y., et al. “Intra- and extra-cranial BCOR-ITD tumours are separate entities within the BCOR-rearranged family”. Journal of Pathology: Clinical Research 3 (2022): 217-232.
4. Arai Y., et al. “Rare Case of BCOR::CCNB3 Sarcoma of Bone”. American Journal of Case Reports 24 (2023): e938158.
5. Kommoss FKF., et al. “Endometrial Stromal Sarcomas with BCOR Internal Tandem Duplication and Variant BCOR/BCORL1 Rearrangements Resemble High-grade Endometrial Stromal Sarcomas with Recurrent CDK4 Pathway Alterations and MDM2 Amplifications”. American Journal of Surgical Pathology 8 (2022): 1142-1152.
6. Image 1: Courtesy: Pathology outlines.
7. Image 2: Courtesy: Cureus.com.