Adenoid cystic carcinoma of the lacrimal gland – A major review

Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare orbital neoplasm. Despite this, it remains the most common epithelial malignancy of the lacrimal gland. It is notorious for high rates of local recurrence, metastases, and mortality despite aggressive management. The treatment protocols range from orbital exenteration or an excision biopsy followed by adjuvant therapy in the form of radiotherapy or chemotherapy or sometimes both. Older studies suggest that orbital exenteration may result in better local control of disease and possibly better long-term survival. This outlook has been challenged by recent studies which suggest multimodal treatment combining various treatment strategies aiming for globe salvage and better disease-free survival. The present review analyzes in detail the various clinical and histopathological features, staging of the disease, management modalities, and treatment outcomes of ACC of the lacrimal gland published over the past 30 years.