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.