Intracranial response to a combination of bevacizumab and epirubicin for an adenoid cystic carcinoma of the external auditory canal: A case report and review of the literature

Abstract Adenoid cystic carcinoma of the external auditory canal is very rare tumour. Its course is mainly indolent, and is characterised by intracranial extension, high rates of loco-regional relapse, and distant metastasis, which culminates in a slow detrimental disease course, eventually leading to death. The main therapeutic modalities are local therapies such as surgery and/or radiotherapy. Systemic treatment has never achieved good clinical outcomes in this setting. Here, we describe the case report of a 50-year-old female, diagnosed with an adenoid cystic carcinoma of the right external auditory canal in 2003. The patient developed multiples recurrences, including an extensive intracerebral progression associated with radio necrosis, for which systemic therapy with bevacizumab and epirubicin was initiated. This achieved an intracerebral partial response with clinical benefit, allowing the rapid tapering of corticosteroid treatment. This case report highlights epirubicin in combination with bevacizumab as a therapeutic option for heavily treated patients with adenoid cystic carcinoma and intracranial involvement.