Chordoma Foundation


Chondroitin sulfate proteoglycan 4 (CSPG4) is a proteoglycan that plays a critical role in cell migration and proliferation.

Location: Chromosome 15q24.2

CSPG4 is a cell-surface protein that is well-expressed on numerous types of cancer cells, including chordoma, but not highly expressed on normal cells. CSPG4 play a roles in tumor cell growth and survival and could be a potential therapeutic target for antibody immunotherapy.

CSPG4 in Chordoma

CSPG4 (also known as neuron-glial antigen 2, NG2, and high molecular weight-melanoma associated antigen, HMW-MAA) is key component of cell migration and proliferation. CSPG4 has been associated with disease severity in other tumors. Expression of CSPG4 could be a useful prognostic biomarker in chordoma. As CSPG4 is expressed on the cell-surface, it is also an attractive candidate for targeting by a CSPG4-specific antibody.

Molecular Evidence

Gene Expression

  • CSPG4 is over expressed in chordoma patient tissue compared to soft tissue sarcomas.1

Protein Expression

  • Immunohistochemical staining of CSPG4 in one cohort found 13/21 (62%) of chordoma cases and 40/84 (48%) of chondrosarcoma lesions were positive. The staining was not correlated with either histologic grade or dedifferentiation.1
  • A retrospective review of 86 patients measured expression of CSPG4 by immunohistochemical staining. 62/82 patient tumors (72%) and 0/6 notochord lesions stained positive for CSPG4. CSPG4 protein expression was significantly associated with increased mortality after adjustment for stain positivity, patient age, sex, and surgical margins (HR 2.31; 95% CI 1.04, 5.17; p=.04). CSPG4-positive tumors were also associated with an increased risk of metastatic disease.2


Schwab J, Boland P, Agaram N, et al. Chordoma and chondrosarcoma gene profile: implications for immunotherapy. Cancer Immunol Immunother. 2009;58(3):339-349.
Schoenfeld A, Wang X, Wang Y, et al. CSPG4 as a prognostic biomarker in chordoma. Spine J. 2016;16(6):722-727.

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