Despite the fundamental role of the TCR pathway in maintaining genomic integrity, individuals with CS have a paradoxically low incidence of cancer. This is intriguing because defective DNA repair mechanisms generally predispose individuals to cancer. The underlying reason for this anomaly is still under active investigation. Some hypotheses suggest that the severe cellular dysfunctions and premature aging associated with CS might limit the lifespan of potentially cancerous cells, thereby reducing the risk of malignancy.
