PTEN and Cowden disease: It is likely that other entities characterized by similar germline PTEN mutationsincluding: (i) Lhermitte-Duclos disease (hamartomatous cerebellar dysplasticgangliocytoma); (ii) Bannayan Riley Ruvacalba syndrome (pediatric hamartomatoussyndrome displaying multiple subcutaneous lipomas, hemangiomas, intestinal polyps andmacrocefaly); and (iii) segmentar forms of a Proteus-like syndrome, constitute, withthe Cowden's syndrome, poles of a symptomatic spectrum depending of germline PTENmutations and generically designated as PTEN Hamartoma Tumor Syndrome(PHTS).15,16