PTEN hamartoma tumor syndrome (PHTS) is caused by germline autosomal-dominant mutations of the tumor suppressor gene PTEN. PHTS encompasses different syndromic disorders including Cowden syndrome (CS), Bannayan Riley Ruvalcaba Syndrome (BRRS), Lhermitte Duclos Syndrome, Proteus and Proteus like syndrome. This evidence concerns the gene PTEN and PTEN hamartoma tumor syndrome.