Cowden's syndrome affecting the mouth, gastrointestinal, and central nervous system: a case report and review of the literature.
Cowden's syndrome (CS) is a rare genodermatosis, of autosomal dominant inheritance and variable phenotype, principally characterized by the formation of hamartomas in various organs, including skin, thyroid, breast, brain, and gastrointestinal tract and by the increased risk for the development of malignancy. The present report details the features of a very rare presentation of Cowden's syndrome affecting not only the mouth and gastrointestinal mucosa but also the central nervous system.
Leão
Jair C
JC
Departamento de Clínica e Odontologia Preventiva, Universidade Federal de Pernambuco, Recife, Brazil. jleao@ufpe.br
Batista
Virgínia
V
Guimarães
Patrícia B
PB
Belo
Josemir
J
Porter
Stephen R
SR
D000328:Adult; D001932:Brain Neoplasms / Q000209:etiology; D006223:Hamartoma Syndrome, Multiple / Q000150:complications* / Q000473:pathology*; D006392:Hemangioma, Cavernous / Q000209:etiology; D006801:Humans; D007417:Intestinal Polyps / Q000209:etiology; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology*; D009062:Mouth Neoplasms / Q000209:etiology* / Q000473:pathology