Abstract
Cowden Syndrome is an autosomal dominant disorder characterized by the development of several hamartomas in a variety of tissues, including the skin, gastrointestinal tract, adipose tissue, and bone and connective tissues. This syndrome is associated with a high risk of developing malignancies, especially breast, thyroid and endometrium cancers. This report presents a case of a 53-year-old patient who sought assistance at the Dentistry Department of the University Hospital of Brasilia (HUB) to receive basic dental treatment. The current and past medical history and oral and facial manifestations led to the diagnosis of Cowden syndrome. This case report aims to highlight the importance of a dental surgeon in the diagnosis of this syndrome, based on the observation of oral manifestations and medical history.KeywordsCowden Syndrome; Multiple Hamartoma Syndrome.
Highlights
Cowden Syndrome is an autosomal dominant disorder characterized by the development of several hamartomas in a variety of tissues, including the skin, gastrointestinal tract, adipose tissue, and bone and connective tissues
The prevalence of Cowden Syndrome is estimated at 1/200.000 [4,5,6] and it is usually diagnosed during the third decade of life, it can present its first signals between 4-75 years of age [5,7]
A 53-year-old male came to the Dentistry Department of the University Hospital of Brasilia (HUB) to receive basic dental treatment
Summary
Cowden Syndrome is an autosomal dominant disease with a wide degree of penetration and variation of expressiveness. It is a rare disorder that predisposes to the development of malignancies such as breast, thyroid and endometrial cancers as well as benign hamartomatous overgrowth of tissues, especially skin, colon and thyroid [2,3,4,5] This syndrome, known as Multiple Hamartoma Syndrome, was first described in 1963 by Lloyd and Denis, with reference to the patient Rachael Cowden [5,6]. After confirming the diagnosis based on established criteria, the patient was asked about the syndrome, referred for evaluation and conduct to the dermatology clinic He continues to monitor his health condition with an oncologist to treat the thyroid cancer and track other possible malignancies
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
