Adenoid cystic carcinoma presenting with bilateral orbital extension from the soft palate.

Adenoid cystic carcinoma (ACC) is a rare neoplasm of secretory epithelium that most commonly occurs in the fifth and sixth decades of life. It is characterized by high recurrence rates and poor response to chemotherapy, In the orbit, ACC usually presents as a lacrimal gland mass. We describe the rare case of a 70-year-old woman who presented with pain during mastication and bilateral facial numbness in the cranial nerve V2 distribution. She was found to have adenoid cystic carcinoma involving the orbits bilaterally without lacrimal gland involvement and without a clear primary tumor. Imaging suggested that the tumor arose from the soft palate by extension along cranial nerves V2 and V3. The patient was treated with radiation therapy with some degree of radiographic improvement 27 months after diagnosis. This case emphasizes the importance of considering adenoid cystic carcinoma when evaluating orbital tumors sparing the lacrimal gland. We also suggest the possibility of an oropharyngeal source with anterograde intracranial extension in cases of putative primary orbital ACC without lacrimal gland involvement.