Abstract
Lacrimal gland adenoid cystic carcinoma (AdCC) is associated with an aggressive clinical course and grave prognosis. A high grade transformation within adenoid cystic carcinoma of lacrimal gland is a rare condition which is even more locally aggressive with frequent neck and distant metastasis. We present a case of left lacrimal gland adenoid cystic carcinoma with high grade transformation to adenocarcinoma NOS type presenting with orbital pain and proptosis. After thorough evaluation for locoregional and distant spread of the disease, the patient underwent left orbital exenteration with orbitectomy and neck dissection with free flap reconstruction. Patient received adjuvant radiation therapy and is presently disease free for last 6 months. A multi-modality management protocol involving surgery, radiotherapy and chemotherapy has been proposed for management of lacrimal gland AdCC with high grade transformation. We report the 4th case in the literature of lacrimal gland adenoid cystic carcinoma with high grade transformation.