Abstract:AIM: To determine the types and incidence of tumors affecting the orbit, conjunctiva and lacrimal glands and to study the trend line of these tumors in the United States from 1973 to 2009. METHODS: We used the publicly available Surveillance, Epidemiology and End Results (SEER) database registries to determine the incidence rates. Age was adjusted to the 2000 US Standard Population. Patients were stratified according to age group, gender, race and histological grouping of tumor lesions. Three age groups were defined: 0-19, 20-49 and ≥50y. Annual percentage changes were calculated to examine trends. RESULTS: The overall age adjusted incidence rate was 3.39 (95%CI: 3.27-3.52) per million person-years. The tumors were more prevalent in age group ≥50 counting 9.51 (95%CI: 9.11-9.92) per million person-years. Most of the soft tissue sarcomas occurred in the young age with incidence rate of 0.35 (95%CI: 0.28-0.42) per million person-years. Lymphomas were the dominant subtype in the adult population with incidence rate of 5.74 (95%CI: 5.43-6.06) per million person-years. Incidence rates were higher in males than females with an overall rate ratio of 1.31 (95%CI: 1.21-1.41) mainly caused by the increase in carcinoma subtypes. White race had a higher tumor incidence with a rate ratio of 1.47(95%CI: 1.25-1.73) driven by the higher incidence of most histological subtypes. Orbital tumors showed a higher incidence rate followed by conjunctival and lacrimal gland tumors with incidence rates of 1.59, 1.37 and 0.43 per million person-years respectively. The trend line of overall incidence of tumors showed a significant increase (APC=3.11, 95%CI: 2.61-3.61) mainly due to increase of lymphomas. This increase was higher than the increase of lymphomas at other sites. CONCLUSION: Orbital, conjunctival and lacrimal gland malignant tumors differ among children and adults. Over the years there has been a noticeable increase in incidence rates of orbital and lacrimal gland tumors mainly caused by an increase in lymphomas and an apparent increase due to advances in diagnostic techniques. ICD-O-3 topographical coding should be improved to consider the different orbital bones and ocular structures.