ORAL MANIFESTATIONS OF THE COWDEN SYNDROME: CASE REPORT

Abstract

Cowden syndrome (CS) is an autosomal dominant disease with increased risk of breast, thyroid, and endometrial malignant neoplasms as well as mucocutaneous lesions. A 63-year-old woman with previous diagnosis of CS was referred to our oral diagnostic clinic complaining of a painless nodule on the left buccal mucosa with history of trauma. The patient also had a history of multiple facial trichilemmomas, palmoplantar keratosis, gastrointestinal polyps, hemangioma on the left upper limb, liposarcoma in the inframammary region, abdominal dermatofibrosarcoma protuberans, and follicular adenoma in the thyroid. Oral examination revealed an isolated painless normochromic nodule on the buccal mucosa and multiple painless normochromic papules diffusely distributed on the upper and lower gingiva and alveolar mucosa. The histopathologic analysis revealed fibrous focal hyperplasia of buccal mucosa nodule and fibroepithelial hyperplasia of upper gingiva. The gingival lesions are one of the oral manifestations of CS. The follow-up has been conducted without oral alterations. Cowden syndrome (CS) is an autosomal dominant disease with increased risk of breast, thyroid, and endometrial malignant neoplasms as well as mucocutaneous lesions. A 63-year-old woman with previous diagnosis of CS was referred to our oral diagnostic clinic complaining of a painless nodule on the left buccal mucosa with history of trauma. The patient also had a history of multiple facial trichilemmomas, palmoplantar keratosis, gastrointestinal polyps, hemangioma on the left upper limb, liposarcoma in the inframammary region, abdominal dermatofibrosarcoma protuberans, and follicular adenoma in the thyroid. Oral examination revealed an isolated painless normochromic nodule on the buccal mucosa and multiple painless normochromic papules diffusely distributed on the upper and lower gingiva and alveolar mucosa. The histopathologic analysis revealed fibrous focal hyperplasia of buccal mucosa nodule and fibroepithelial hyperplasia of upper gingiva. The gingival lesions are one of the oral manifestations of CS. The follow-up has been conducted without oral alterations.