Alterations in the phosphatase and tensin (PTEN) homologue gene result in PTEN hamartoma tumor syndrome, which includes Cowden, Bannayan-Riley-Ruvalcaba, and PTEN-related Proteus and Proteus-like syndromes. Pathogenic variants in the PTEN gene are associated with increased risk for developing multiple benign and malignant tumors, some of which may occur in childhood.