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

Area of Science:

• Oncology
• Dermatology
• Otolaryngology

Background:

• Extramammary Paget's disease (EMPD) is a rare intraepithelial adenocarcinoma.
• Typically affects apocrine sweat gland-rich areas like the anogenital region and axillae.
• Rarely occurs in non-apocrine sites, posing diagnostic and therapeutic challenges.

Purpose of the Study:

• To report a rare case of EMPD involving the external auditory canal.
• To document extensive temporal bone involvement and skull base invasion.
• To discuss the clinical presentation and management of this rare entity.

Main Methods:

• Case presentation of a 40-year-old male with a lesion in the external auditory canal.
• Diagnosis confirmed via incisional biopsy and immunohistochemical analysis.
• Assessment of tumor resectability and treatment planning.

Main Results:

• The patient presented with a vegetating lesion, facial nerve paralysis, and hearing loss.
• Histopathological and immunohistochemical analyses confirmed EMPD.
• The tumor was deemed inoperable due to extensive skull base invasion.

Conclusions:

• This represents the third reported case of EMPD in the external auditory canal.
• Prognosis for EMPD in this location appears unfavorable, irrespective of treatment.
• Highlights the importance of considering rare diagnoses in unusual locations.