Adenoid Cystic Carcinoma

A patient with past medical history of a previously resected lacrimal gland mass presented to the emergency room with worsening unilateral proptosis and eye pain with persistent new eversion of the upper eyelid for several days. Examination was significant for severe proptosis of the right eye, chemosis, stromal haze. Best corrected visual acuity (BCVA) of the right eye was light perception. On further history taking, it was noted that the patient had their mass resected seven years prior by neurosurgery who was not aware of the diagnosis of adenoid cystic carcinoma until frozen sections were obtained intraoperatively. The patient subsequently was evaluated for management of ACC by ophthalmology but was lost to follow-up for several years. After re-presentation to the emergency department, the patient underwent enucleation and is now undergoing evaluation for systemic chemotherapy.

Lacrimal gland ACC is a disease with high mortality when treated with the standard of care conventional therapy of surgery and radiation. Long-term data from IACC treatment protocols demonstrate significantly improved mortality rates with promisingly high rates of disease-free survival. 

Presentation Date: 12/17/2020

Issue Date: 07/23/2021