Chordoma Foundation

B7-H3 (CD276)

B7-H3 (also known as CD276) is an immune checkpoint receptor that plays an important role in cancer cell migration, invasion, and proliferation.

Location: Chromosome 15q24.2

B7-H3 is a member of the B7/CD28 superfamily of immune checkpoint molecules. Overexpression of B7-H3 in numerous cancer types has been shown to be correlated with advanced tumor stage and high tumor grade. B7-H3 has possible roles in regulating cancer metabolism, protein regulation, cell proliferation, and epithelial-mesenchymal transition.

B7-H3 in Chordoma

  • B7-H3 (CD276 antigen) is key component of cell migration and proliferation. Expression of B7-H3 could be a useful prognostic biomarker in chordoma. As B7-H3 is expressed on the cell-surface and is unregulated on tumor compared to normal cells, it also represents an attractive candidate for targeting by antibody immunotherapy in bone cancers.1
  • Molecular Evidence

Molecular Evidence

Protein Expression

  • 58/70 (83%) chordoma cases were positive for B7-H3 expression by immunohistochemistry. 1 of 6 notochord controls stained positively for B7-H3.2
  • Protein expression of B7-H3 was not associated with metastasis or survival but had some association with local recurrence3 and lack of expression of B7-H3 was suggestive of a benign course.2

He L, Li Z. B7-H3 and its role in bone cancers. Pathol Res Pract. 2019;215(6):152420.
Schoenfeld AJ, Nielsen GP, Rosenberg AE, et al. Immunohistochemical analysis Of chordoma targeting B7H3 and HMW-MAA. The Orthopaedic Journal at Harvard Medical School. Published 2018. Accessed 2019.
Schoenfeld A, Nielsen GP, Hornicek F, Duan Z, Ferrone S, Schwab JH. CSPG4 and B7-H3 as prognostic biomarkers in chordoma. JCO. 2010;28(15_suppl):10050-10050. doi:10.1200/jco.2010.28.15_suppl.10050

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