Adenoid cystic carcinoma of the lacrimal gland: A rare case report with literature review

Adenoid cystic carcinoma (ACC) primarily originates from the minor and major salivary glands of the head and neck regions. ACC affecting the eye and orbit (including the lacrimal gland, sac, and duct) was determined to account for about 1.8% of all cases. A poor overall prognosis and a tendency for local recurrence and metastasis despite vigorous therapy characterize lacrimal gland ACCs. There are no clear-cut guidelines for treating these rare tumors in unusual places, but recent literature suggests orbital excision with or without bone removal instead of globe-sparing resection and adjuvant radiation therapy. In this case study, we show an unusual case of an ACC in the left superolateral orbit with the demonstration of clinical, radiological, morphology, and immunohistochemistry findings along with management protocols and a review of the relevant literature.