A Case of Adenoid Cystic Carcinoma of the Ectopic Lacrimal Gland

ABSTRACT

Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare malignant tumor accounting for 1.6% of all orbital tumors. We report a case of ectopic adenoid cystic carcinoma that developed on the nasal orbital side of the face adjacent to the nasal cavity. A 60-year-old man developed progressive right ocular proptosis and pain for over three years. The orbit MRI and CT revealed a 24x36x17 mm-sized lower signal mass on the medial side of the right medial rectus muscle. It also showed an erosion of the orbital bone in the superior and medial orbital walls. An endoscopic biopsy was performed and a frozen section of the tissue in the operating room showed no evidence of a malignancy. The mass was excised by endoscopic surgery. The final pathology was an adenoid cystic carcinoma. An orbital exenteration was recommended but the patient refused the procedure and, after receiving chemotherapy and radiation therapy, his condition is being followed. Some of the orbital mass remains in the posterior orbital area immediately after the endoscopic excision. The patient underwent his first session of Cyberknife radiosurgery therapy after concurrent chemotherapy and radiotherapy.