Dyskeratosis congenita (DC) encompasses a large spectrum of diseases and 
clinical manifestations generally related to premature aging, including bone 
marrow failure and cancer predisposition. The major risk factor for DC is to 
carry germline telomere-related mutations - in telomerase or telomere shelterin 
genes - which results in premature telomere dysfunction, thus increasing the 
risk of premature aging impairments. Despite the advances that have been 
accomplished in DC research, the molecular aspects underlying the phenotypic 
variability of the disease remain poorly understood. Here different aspects of 
telomere biology, concerning adult stem cells senescence, tumor suppression and 
cancer are considered in the context of DC, resulting in two translational 
models: late onset of DC symptoms in telomere-related mutations carriers is a 
potential indicator of increased cancer risk and differences in tumor 
suppression capacities among the genetic subgroups are (at least partial) causes 
of different clinical manifestations of the disease. The limitations of both 
models are presented, and further experiments for their validation, as well as 
clinical implications, are discussed.