Adenoid Cystic Carcinoma of the Lacrimal Gland

To explore the various aspects of adenoid cystic carcinoma of the lacrimal gland (LACC), including its clinical features, presentations, genetic factors, as well as current and potential future treatment options.

A thorough exploration of the literature was conducted by extensively searching the PubMed database. A total of 68 articles with a primary focus on adenoid cystic carcinoma were selected.

LACC is the most frequent malignant epithelial tumor of the lacrimal gland. It is characterized by the symptoms such as pain, progressive swelling of the eye, double vision, and bulging of the eye. Although LACC is a rare tumor, early detection and appropriate treatment are crucial due to its aggressive nature which includes high recurrence rate and a significant risk of mortality. The primary treatment options for LACC involve a wide range of surgical procedures, including orbitotomy, exenteration, and cranio-orbital resection. Neoadjuvant intra-arterial chemotherapy represents the paradigm shift in the treatment of LACC enhancing patient survival when compared to conventional treatments.

The existing literature emphasizes the importance of identifying prognostic factors that can guide treatment decisions and enhance the outcomes for patients with LACC.