Adenoid Cystic Carcinoma of the Nasopharynx

Background: Adenoid cystic carcinoma of the nasopharynx is rare, and there are few articles that describe its clinical characteristics, treatment, and prognosis. We set out to describe our institution’s experience with nasopharyngeal adenoid cystic carcinoma.

Methods: A retrospective review of the clinical charts of patients who were diagnosed with adenoid cystic carcinoma of the nasopharynx from 1984 to 2006. Data on the patient demographics, clinical presentation, stage, management, and treatment outcomes were collected.

Results: Eighteen cases with adenoid cystic carcinoma of the nasopharynx were diagnosed from 1984 to 2006. The median follow-up was 52.5 months. The male-to-female ratio was 10:8, with age of presentation ranging from 28 to 79 years (median age 51.5 years). The majority of the patients presented with T4 disease (61.1%), 27.8% presented with T1 disease, and 11.1% with T2 disease. Only 2 of the 18 patients (11.1%) had nodal metastasis at presentation, and none had distant metastasis at presentation. Patients with T1/2 disease were treated with surgery and adjuvant radiation, except for one patient who had radiation alone. In patients with T4 disease, those who were diagnosed before year 2000 were treated with surgery and adjuvant radiation or radiation only. After year 2000, all patients with T4 disease were treated with concurrent chemoirradiation. At 5 years follow-up, all T1/T2 patients were alive, whereas all T4 patients were dead or alive with disease . The median survival of T4 patients was 53 months, with a range of 19 to 121 months. Of the T4 patients, 72% responded to initial treatment, and the median disease-free survival was 24.5 months

Conclusions: Adenoid cystic carcinoma of the nasopharynx when detected early and treated with surgery and adjuvant radiotherapy appears to have an excellent prognosis, with 100% 5-year disease-free survival in our series. Thus for early disease we recommend surgery with adjuvant radiation. Although the prognosis of patients with T4 disease remains poor, where none were disease free at 5 years follow-up, patients who responded to treatment had a significant disease-free period. We are, however, unable to conclude the best modality of treatment for this group due to the small numbers.