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Lacrimal gland adenoid cystic carcinomas are rare, aggressive orbital tumors characterized by poor overall prognosis, tendency for local recurrence and metastasis despite aggressive treatment. Treatment continues to be controversial. Many authorities today will often initiate surgery (orbital exenteration with or without bone removal vs. globe-sparing resection) and adjuvant radiotherapy (external beam or proton beam therapy). We introduce a case of lacrimal gland adenoid cystic carcinoma treated with orbital exenteration and adjuvant volumetric modulated arc therapy, and discuss the related literature.

Lacrimal gland adenoid cystic carcinoma: report of an unusual case with literature review