Malignant epithelial tumors of the lacrimal gland: a clinicopathologic study of 21 cases.

OBJECTIVE: To analyze 120 lacrimal gland masses collected during a 23-year period in the Ophthalmic Pathology Laboratory at the Cullen Eye Institute, Houston, Tex.

METHODS: Of the 120 lacrimal gland tumors, we focused on a detailed clinicopathologic study of 21 malignant epithelial tumors. Follow-up was available in 19 (90%) of 21 cases.

RESULTS: About two thirds of the masses (75 cases) were inflammatory, one third (41 cases) were of epithelial origin, and 3% (4 cases) were malignant lymphomas. Of the epithelial tumors, 17 (41%) were benign mixed tumors, 12 (29%) were adenoid cystic carcinomas arising de novo, 2 (5%) were adenocarcinoma arising de novo, 7 (17%) were malignant mixed tumors, and 3 (7%) were metastatic carcinoma. All of the patients with adenoid cystic carcinoma had local recurrences, and 60% of the patients died of their tumors (mean survival, 5 years); only 2 patients were alive 13 and 16 years after the initial surgery, both of whom had radical surgical procedures for recurrence following orbital exenteration.

CONCLUSION: The histopathologic classification and management of these tumors are discussed. This study supports the dismal prognosis of adenoid cystic carcinoma arising de novo.