Ceruminous adenocarcinoma is a rare malignant neoplasm of the glandular structures of the external auditory canal. The true incidence and behavior of these rare tumors are still unclear due to confusing terminology, classification and histological definitions. Therefore, the ENT surgeon faces major difficulties in choosing the method of management – conservative or more radical surgery – with the addition or not of radiotherapy. We report a 57-year-old male patient with a recurrence of a previously excised (maybe partially) and irradiated ceruminous adenocarcinoma of the right external auditory canal. Aggressive surgery was considered as the treatment of choice. However, the patient refused this approach and, as a consequence, a conservative excision was performed but with histologically confirmed healthy margins. To our surprise, the patient showed an excellent response and he is disease free 3 years following the last operation. Although recurrences usually occur within months after inadequate management, some may happen even 7 years post treatment. Therefore, routine long-term follow-up was advised.
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