Abstract
IntroductionExtramammary Paget’s disease is a rare histological type of intraepithelial adenocarcinoma that mainly affects apocrine sweat gland-rich areas. Predilection sites include the anogenital region and, less commonly, the axillae. These tumors rarely occur in non-apocrine regions. The aim of this case report is to describe a case of extramammary Paget’s disease in the external auditory canal with extensive temporal bone involvement and skull base invasion.Case presentationA 40-year-old Caucasian man presented with a progressively growing, vegetating lesion localized in his right-hand external auditory canal. He had peripheral facial nerve paralysis and complete ipsilateral hearing loss. An incisional biopsy suggested extramammary Paget’s disease, and the immunohistochemical analysis confirmed the diagnosis. The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.ConclusionWe report here the third case of extramammary Paget’s disease affecting the external auditory canal to be described in the literature. In all the three cases, the prognosis was unfavorable despite treatment.
Highlights
Extramammary Paget’s disease is a rare histological type of intraepithelial adenocarcinoma that mainly affects apocrine sweat gland-rich areas
We report here the third case of extramammary Paget’s disease affecting the external auditory canal to be described in the literature
Despite the presence of modified ceruminous sweat glands in the external auditory canal, there have been only two previously reported cases of Extramammary Paget’s disease (EMPD) in this region [2,3]. The aim of this case report is to describe the case of a patient with EMPD in the external auditory canal, with extensive temporal bone involvement and skull base invasion
Summary
We report here the third case of extramammary Paget’s disease affecting the external auditory canal to be described in the literature.
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