Based on current NCCN guidelines, the recommended eligibility criterion to genetically test individuals for the presence of PVs in genes (BMPR1A, PTEN, SMAD4, and STK11) associated with hamartomatous polyposis syndromes (JPS, PJS, or PHTS) is the occurrence of at least two GI hamartomatous polyps [12]. This evidence concerns the gene SMAD4 and juvenile polyposis syndrome.