18435 Extramammary Paget disease of the eyelid with clivus malignancy

Abstract

Extramammary Paget disease (EMPD) was originally described in 1889 by Crocker, who reported genital lesions histologically similar to those described by Paget in 1874. EMPD is a rare intraepithelial adenocarcinoma that affects body parts with abundant apocrine glands. Primary form is the most common, originating in the epidermis or cutaneous adnexa. Secondary form, less often, occurs in association with an underlying malignancy, usually of the lower gastrointestinal or urinary tract. EMPD is an uncommon neoplasm, which occurs mostly in the anogenital region, but can arise in other areas of skin or mucosa. It comprises 2% or less of primary vulval neoplasms and is even rarely in other sites. There are reports of EMPD arising in other areas, such as axilla and ear. The first description of EMPD of the eyelid was made by Hagedoorn in 1937, related with previous trachoma and posterior developing of eyelid carcinoma. After that, Whorton described in 1955 a case of EMPD of the eyelid related to carcinoma of Moll glands. The purpose of this report is to describe a case of EMPD of the eyelid in a 60 year female patient, who was also diagnosed with clivus malignancy. She presented an erythematous, exulcerated and squamous lesion on the ocular area, eyelids and cheek. Histologic analysis showed EMPD disease from different samples.There were scars, caused by previous surgeries, and an eye prosthesis after the treatment of clivus malignancy. Besides the lesion extension, she also presents undefined intracranial lesions and the treatment plan is a challenge.