It is currently unclear what the gold standard is for early cancer detection programs in children with cancer predisposition syndromes stemming from gene mutations in the germline, such as Li-Fraumeni (TP53), Von Hippel Lindau (VHL), familial adenomatous polyposis (APC), DICER1 syndrome or subtypes of primary immunodeficiencies [34]. The gene discussed is APC; the disease is DICER1-related tumor predisposition.