The tumor is strongly associated with TP53-related tumor syndromes, such as Li–Fraumeni syndrome, particularly in Southern Brazil due to the inherited TP53p.R337H germline mutation, which significantly increases tumor incidence in this region (Ribeiro et al. 2001, Pinto et al. 2004, Wasserman et al. 2015). The gene discussed is TP53; the disease is neoplasm.