A comparison of the demographics, clinical features, and survival of patients with adenoid cystic carcinoma of major and minor salivary glands versus less common sites within the Surveillance, Epidemiology, and End Results registry.

BACKGROUND

The scientific literature to date lacks population-based studies on the demographics, clinical features, and survival of patients with adenoid cystic carcinoma (ACC) of different anatomic sites.

METHODS

The authors identified 5349 patients who had ACC of the major salivary glands (N = 1850), minor salivary glands (N = 2077), breast (N = 696), skin (N = 291), lung and bronchus (N = 203), female genital system (N = 132), and eye and orbit (N = 100) from the Surveillance, Epidemiology, and End Results (SEER) registry. Differences in demographics, clinical features, and survival of patients were assessed.

RESULTS

ACC of the eye and orbit was associated with younger age at presentation (mean age,49.9 years). ACC of the skin or breast tended to present with less aggressive prognostic features, whereas ACC of the lung and bronchus or eye and orbit tended to present with more aggressive prognostic features. In a multivariate survival analysis of patients who presented with localized disease, patients with ACC of the breast (hazard ratio [HR], 0.40) or skin (HR, 0.40) had a significantly lower risk death than patients with ACC of the major salivary glands; whereas patients with ACC of the lung and bronchus (HR, 3.72) or the eye and orbit (HR, 3.67) had a significantly higher risk. For patients who presented with regional disease, the only clear prognostic difference in multivariate analysis was that patients with ACC of skin fared significantly better.

CONCLUSIONS

The demographics and clinical features of patients with ACC differ by disease site. The current results indicated that site may be an important predictor of survival for patients who present with localized disease but is less important for patients who present with regional disease.