Chordoma Foundation


Survivin, baculoviral IAP repeat-containing 5 (BIRC5), is a protein that inhibits apoptosis (programmed cell death) and is highly expressed in many cancers.

Cancers expressing survivin are characterized by increased resistance to chemotherapy, increased likelihood of recurrence, and reduced patient survival.1 Since the expression of survivin contributes to the survival of cancer cells, targeting this molecular player has been explored in anti-cancer therapy.

Survivin in Chordoma

In light of high survivin expression in numerous tumor types and the correlation of high expression with poor prognosis,  researchers have begun to explore survivin’s role in chordoma. Though study numbers are still low, high survivin expression has been detected in many chordoma samples and studies continue to evaluate its prognostic significance. This page contains a summary of published research evaluating survivin inhibition as a potential treatment for chordoma.

Molecular Evidence

Gene Expression

Surviving expression was higher in recurrent chordomas compared to primary chordoma cases. A multivariate Cox proportional-hazards model suggested that survivin expression level was an independent prognostic factor for tumor progression. Survivin expression level was also an independent prognostic factor for death.2

Protein Expression

Chen et al. (2013) found that 21 of 30 chordoma samples were positive for survivin and, survivin expression was significantly associated with recurrence.3 A more recent study by Froehlich et al. (2015) found surviving expression in 22 of 34 primary samples and 16 of 16 recurrent samples.4

Preclinical Evidence

In-vitro Efficacy

  • siRNA knockdown: Knockdown led to significant changes in the cell cycle distrbituions of chordoma cell lines MUG-Chor1 and U-CH1.4
  • YM155: Treatment of chordoma cell lines MUG-Chor1 and U-CH1 with survivin suppressant YM155 inhibited cellular proliferation, decreased survivin mRNA and protein expression, and increased celluar apoptosis.4
Fukuda S, Pelus L. Survivin, a cancer target with an emerging role in normal adult tissues. Mol Cancer Ther. 2006;5(5):1087-1098. [PubMed]
Ma J, Tian K, Du J, Wu Z, Wang L, Zhang J. High expression of survivin independently correlates with tumor progression and mortality in patients with skull base chordomas. J Neurosurg. January 2019:1-10.
Chen C, Yang H, Chen K, et al. High expression of survivin in sacral chordoma. Med Oncol. 2013;30(2):529. [PubMed]
Froehlich E, Rinner B, Deutsch A, et al. Examination of survivin expression in 50 chordoma specimens–A histological and in vitro study. J Orthop Res. 2015;33(5):771-778. [PubMed]

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