Extramammary Paget’s disease affecting the external auditory canal: a case report

Journal of Medical Case Reports

Francisco Monteiro de Castro¹  Ana Maria da Silva Castro³  Jônatas Catunda de Freitas⁴  Igor Moreira Veras²  Walber de Oliveira Mendes⁴  Francisco de Assis Castro Bomfim¹ 

[1] Department of Head and Neck Surgery, Walter Cantídio Hospital, 608 Prof. Costa Mendes Street, Rodolfo Teófilo, Fortaleza, Ceará ZIP Code: 60430-140, Brazil;Radiotherapy Department, Regional Center for Integrated Oncology (CRIO), 1300 Francisco Calaça Street, Fortaleza, Ceará ZIP Code: 60336-550, Brazil;Livino Pinheiro Laboratory, Haroldo Juaçaba Hospital, Cancer Institute of Ceará (ICC), 1222 Papi Junior Street, Fortaleza, Ceará ZIP Code: 60430-230, Brazil;Head and Neck Surgery Research Group, Faculty of Medicine, Federal University of Ceará, 608 Prof. Costa Mendes Street, Rodolfo Teófilo, Fortaleza, Ceará ZIP Code: 60430-140, Brazil

关键词: Paget’s disease;    Head-neck tumors;    Extramammary;    External ear;    External auditory canal;   

Others  :  825076
DOI  :  10.1186/1752-1947-7-250

 received in 2013-04-28, accepted in 2013-09-26,  发布年份 2013

【 摘 要 】

Introduction

Extramammary 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 presentation

A 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.

Conclusion

We 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.

【 授权许可】

   
2013 Bomfim et al.; licensee BioMed Central Ltd.

【 图 表 】

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