Portuguese Version

Year:  1998  Vol. 64   Ed. 6 - (11º)

Relato de Casos

Pages: 631 to 634

Hamartoma Multiple Syndrome (Cowden Syndrome): The Importance of Cutaneous-mucosal Manifestations for Otolaringologist.

Author(s): João J. Maniglia* ;
Cynthia C. S. Nicolau**;
Guilherme S A. Catani***
Márcia L. Brotto**** ,
Ricardo Maniglia *****.

Keywords: multiple hamartoma syndrome, Cowden's disease, hamartoma

Cowden is a dominant autossomic syndrome where hamartomas are present. lts most common manifestations are gastrointestinal tract polyps, thyroid diseases (goiter or neoplasia), breast diseases (fibrocystic disease or neoplasia) and cutaneous-mucosal lesions. The most consistent criterion for diagnosis are hamartomatóus skin or mucosal lesions present in 99-100% of cases. The authors describe a 36 year old female patient with bucal mucosa papilas, thyroid follicular adenoma and infiarnatory polyp of large intestines. They also emphasize diagnosis and adequate follow up of these patients in reference to malignancies of thyroid, breast and intestines.






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