Adenoid cystic carcinomas of the lacrimal gland in childhood and adolescence

Objective: To see if there is a correlation between histologic features of these tumors and final outcome. Design: A small series of cases of adenoid cystic carcinomas of the lacrimal gland in patients 18 years of age or younger were evaluated. Participants: A total of 11 cases of 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: Histologic material obtained by excision of lacrimal gland tumors was evaluated for different morphologic parameters. Clinical follow-up information was reviewed. Main Outcome Measures: All cases were evaluated for proportion of a basaloid histologic pattern: necrosis, hemorrhage, mitotic count, and perineural, vascular, intraosseous, leptomeningeal, and optic nerve invasion. These parameters were examined for an association with the clinical follow-up that was obtained for eight of the patients (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). Survivors had 25% or less basaloid histology. Necrosis, hemorrhage, perineural invasion, and mitotic count were less prominent in survivors than in those who died of disease. Vascular invasion was seen only in fatal cases. Conclusions: Young patients with adenoid cystic carcinomas have a better prognosis than do adult patients, which may be due to their tumors having less aggressive histologic features.