Adenoid Cystic Carcinoma of the Nasopharynx

Adenoid cystic carcinoma of the nasopharynx is a rare disease with 34 previously reported cases in the literature. We report three additional cases with clinical presentation, treatment, and outcome. The presenting symptoms were facial pain in one case, middle ear effusion and epistaxis in another, and diplopia in the third. Radiotherapy was administered with relief of symptoms in all three cases. However, two patients with long-term follow-up developed local recurrence as well as distant metastases. The literature review showed that patients with this disease present with symptoms and signs such as insidious onset of facial pain, epistaxis, diplopia, and hearing loss. The final outcome, despite vigorous treatment, was dismal.