EyeWiki:Featured article/April 15, 2020

From EyeWiki

Featured Article for April 14, 2020

Adenoid Cystic Carcinoma of the Lacrimal Gland

Lacrimal gland tumors comprise about 10% of all space-occupying orbital tumors. About 20% of solid lacrimal gland tumors are of epithelial origin, with about 45% being malignant. Of the malignant epithelial lacrimal gland tumors, about 60% are adenoid cystic carcinomas, and these most often involve the orbital lobe of the lacrimal gland.

Adenoid cystic carcinoma generally presents in young or middle-aged adults with a mean age of 40 years, but can occur in the first decade of life. There is a slight predilection for women. Diagnosis of adenoid cystic carcinoma generally occurs within 6 months of presenting signs and symptoms. Given the superotemporal location of the lacrimal gland in the orbit, often the initial presenting sign is proptosis with inferior and nasal deviation of the globe (Fig. 1). Pain from bone and nerve invasion by this unencapsulated tumor is a distinguishing feature from benign lesions, which tend to be slowly progressive without pain. Nerve invasion may also present with frontotemporal hypoesthesia. Patients may also present with “S-shaped” blepharoptosis, diplopia, and ocular motility deficits.