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

Bomfim Fde A, Mendes Wde O, Veras IM, de Freitas JC, Castro AM, de Castro Junior FM - J Med Case Rep (2013)

Bottom Line: He had peripheral facial nerve paralysis and complete ipsilateral hearing loss.The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.In all the three cases, the prognosis was unfavorable despite treatment.

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ABSTRACT

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.

No MeSH data available.


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Clinical features of the patient with extramammary Paget’s disease.
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Figure 1: Clinical features of the patient with extramammary Paget’s disease.

Mentions: The patient was a 40-year-old Caucasian man who presented with a 3-month history of a progressively growing, vegetating lesion localized in his right-hand external auditory canal. Upon physical examination, a 2.5cm bleeding, ulcerated lesion was observed to occlude his right external auditory canal (Figure 1). The patient had already developed peripheral facial nerve paralysis and complete ipsilateral hearing loss.


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

Bomfim Fde A, Mendes Wde O, Veras IM, de Freitas JC, Castro AM, de Castro Junior FM - J Med Case Rep (2013)

Clinical features of the patient with extramammary Paget’s disease.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4226198&req=5

Figure 1: Clinical features of the patient with extramammary Paget’s disease.
Mentions: The patient was a 40-year-old Caucasian man who presented with a 3-month history of a progressively growing, vegetating lesion localized in his right-hand external auditory canal. Upon physical examination, a 2.5cm bleeding, ulcerated lesion was observed to occlude his right external auditory canal (Figure 1). The patient had already developed peripheral facial nerve paralysis and complete ipsilateral hearing loss.

Bottom Line: He had peripheral facial nerve paralysis and complete ipsilateral hearing loss.The tumor was considered inoperable because of the extensive skull base involvement, and he was referred for palliative radiotherapy.In all the three cases, the prognosis was unfavorable despite treatment.

View Article: PubMed Central - HTML - PubMed

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus