ADENOID CYSTIC CARCINOMAS OF THE LACRIMAL GLAND IN CHILDHOOD AND ADOLESCENCE

Objective: To see if there is a correlation between histologic feature s of these tumors and final outcome. Design: A small series of cases o f adenoid cystic carcinomas of the lacrimal gland in patients 18 years of age or younger were evaluated. Participants: A total of 11 cases o f adenoid cystic carcinoma of the lacrimal gland in patients 18 years of age or younger found in the registry of Ophthalmic Pathology at the Armed Forces Institute of Pathology were studied. Intervention: Histo logic material obtained by excision of lacrimal gland tumors was evalu ated for different morphologic parameters. Clinical follow-up informat ion was reviewed. Main Outcome Measures: All cases were evaluated for proportion of a basaloid histologic pattern: necrosis, hemorrhage, mit otic count, and perineural, vascular, intraosseous, leptomeningeal, an d optic nerve invasion. These parameters were examined for an associat ion with the clinical follow-up that was obtained for eight of the pat ients (mean follow-up, 10 years; range, 2-14 years). Results: Most of the patients were female (M:F = 2:9). Mean age was 14 years (range, 6. 5-18 years). Of the patients with follow-up, 5 (62.5%) of 8 survived. Estimated survival rate at 15 years was 58% (Kaplan-Meier analysis). S urvivors had 25% or less basaloid histology. Necrosis, hemorrhage, per ineural invasion, and mitotic count were less prominent in survivors t han in those who died of disease. Vascular invasion was seen only in f atal cases. Conclusions: Young patients with adenoid cystic carcinomas have a better prognosis than do adult patients, which may be due to t heir tumors having less aggressive histologic features.