Epidemiology and treatment of lacrimal gland tumors: a population-based cohort analysis.

IMPORTANCE: Primary tumors of the lacrimal gland are rare and are associated with substantial morbidity and mortality. The literature regarding these tumors is limited to case series and case reports.

OBJECTIVE: To examine the incidence, treatment, and overall survival (OS) and disease-specific survival (DSS) of patients with cancer of the lacrimal gland.

DESIGN, SETTING, AND PARTICIPANTS: Population-based cohort analysis using the Surveillance, Epidemiology, and End Results (SEER) database to identify patients with primary tumors of the lacrimal gland from 1973 to 2010.

MAIN OUTCOMES AND MEASURES: Overall survival and DSS.

RESULTS: A total of 321 patients with nonlymphoid tumors of the lacrimal gland were identified. The most common histological subtypes were adenoid cystic carcinoma (ACC) (32.1%) and squamous cell carcinoma (SCC) (29.9%). Survival analysis revealed a 5-year OS and DSS for all lacrimal gland tumors of 60% and 75%, respectively. On univariate analysis, low tumor grade (P = .04) and surgical treatment (P < .001) were associated with significantly better OS. For ACC tumors, surgery (P = .009), but not radiotherapy (P = .44), was found to significantly improve OS. For SCC tumors, surgical treatment significantly improved both OS (P < .001) and DSS (P = .004); radiation therapy also significantly improved OS (P = .03). Using a multivariable analysis model, age (hazard ratio [HR], 1.03 [95% CI, 1.01-1.04]; P < .001), surgery (HR, 0.43 [95% CI, 0.25-0.75]; P = .003), and T stage at presentation (HR, 1.18 [95% CI, 1.01-1.37]; P = .03) were found to be independent predictors of OS. For ACC alone, age (HR, 1.04 [95% CI, 1.02-1.06]; P < .001) and surgery (HR, 0.35 [95% CI, 0.13-0.91]; P = .03) were independent predictors of OS. For SCC, age (HR, 1.05 [95% CI, 1.02-1.09]; P = .005), surgical resection (HR, 0.31 [95% CI, 0.12-0.83]; P = .02), and radiation therapy (HR, 0.33 [95% CI, 0.14-0.80]; P = .01) were independent predictors of OS.

CONCLUSIONS AND RELEVANCE: Our study demonstrates that ACC is the most common malignant epithelial neoplasm of the lacrimal gland. Determinants of survival for tumors of the lacrimal gland include age at diagnosis and surgical therapy. Radiation therapy is associated with improved DSS in SCC but not in ACC.