PP - MULTIPLE ENDOCRINE NEOPLASIA TYPE 2B IN CHILD: CASE REPORT

Abstract

Multiple endocrine neoplasias are autosomal dominant genetic syndromes. The MEN type 2B is featured by mucosal neuromas with tumoral involvement in thyroid, parathyroid and adrenal. Individuals present marfanoid and dolichocephalic body, narrow face, thick and protruding lips, overbite, anterior diastema, and may have nodular lesions on the tongue and oral mucosa. The patient, S.A.S., female, African American, 5 years, accompanied by her father, appeared in 1FAODO-UFMS suspected of retention cyst or pyogenic granuloma. On examination, there were thin and elongated limbs, café au lait spots on trunk, front and hypertelorism bossa, primary teeth, permanent teeth formation, nodular sessile based on lower gingival mucosa, on region of the 72 to 83 elements, 3 cm, slightly delimited and firm to the touch, subjected to biopsy. Histopathology found numerous circular nerve bundles and discreet perineural thickening in connective tissue. She was diagnosed with multiple endocrine neoplasias, and was referred to the endocrinologist, pediatrician and genetic study. Furthermore the father had the same soft nodules and massive stock pedunculated mass in the posterior side of the thorax, suggesting neurofibromatosis. Thorough intraoral examination and viewing the patient as a whole is essential because oral abnormalities are usually linked to systemic diseases, and early oral diagnosis results will improve life quality, and favorable prognosis.