PHTS patients have diverse phenotypes such as Cowden disease (CS), Bannayan-Riley-Ruvalcaba syndrome (BRRS), PTEN-related Proteus syndrome (PS), and Proteus-like syndromes, but the PHTS designation should be used for all these syndromes, when a germline PTEN mutation is detected [173, 175, 177]. Here, PTEN is linked to Cowden disease.