[Multiple hamartoma syndrome]

We describe the clinicopathological features of multiple hamartoma and neoplasia syndrome (Cowden's syndrome) in a 58 year-old man and in a 56 year-old woman. The typical mucocutaneous lesions of this disorder were present in both cases. Visceral hamartomas involving thyroid and gastrointestinal system (polyposis) were present in both patients. Dermal or submucous fibromas are proposed as an additional diagnostic criterium of Cowden's syndrome. Associated abnormalities and visceral involvement in multiple hamartoma syndrome are reviewed.