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.
Mucoepidermoid carcinoma (MEC) is a common malignant tumor composed of mucinous cells, clear cells and squamoid tumor cells. It is usually localized on the salivary glands, however can very rarely be intraosseous. This tumor affects women more than men and is more common in the mandible. In this report, we present a case of a 45-year-old woman with a painless intraoral mass for 10 years which was repeatedly misdiagnosed, evolving into an extensive lesion. After an appropriate approach, she was diagnosed with MEC of the mandible and submitted to a left hemimandibulectomy accompanied by microsurgical reconstruction with excellent results.
O adenoma pleomórfico é a neoplasia mais comum das glândulas salivares maiores e menores; entretanto, sua variante metastática é rara. Assim, o objetivo desse trabalho é relatar um caso dessa entidade. Paciente feminina de 42 anos realizou uma ressecção transoral de adenocarcinoma de glândulas salivares menores em mucosa jugal esquerda em 2008. Após 8 anos, apresentou recidiva tumoral e metástase para linfonodos submandibulares, sendo esta identificada como adenoma pleomórfico. Paciente foi submetida a ressecção da lesão, com maxilectomia e esvaziamento radical modificado, com reconstrução a partir de retalho do músculo masseter. Paciente foi encaminhada para radioterapia adjuvante e está livre de doença há 1 ano. A metástase dos adenomas pleomórficos se manifestam anos após o tumor primário. O mecanismo fisiopatológico da disseminação é incerto e marcadores tumorais de maior agressividade para esses adenomas estão sendo estudados. Reafirma-se, portanto, a necessidade de excisão completa e tecnicamente perfeita da neoplasia primária.
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