Adenoid Cystic Carcinoma of the Lacrimal Gland

A 43-year-old female presented to her primary care physician with swelling of the lateral aspect of the right upper eyelid without visual complaints. The primary care physician noted proptosis of the right globe and ordered a CT scan which revealed a mass in the right lacrimal gland fossa. The mass was biopsied and a diagnosis of adenoid cystic carcinoma of the lacrimal gland was confirmed by histopathology. PET scanning revealed hypermetabolic foci within the cervical and thoracic vertebrae as well as the ischial tuberosity. Biopsy of the hypermetabolic focus within the ischial tuberosity confirmed metastatic disease. The patient underwent intra-arterial chemotherapy followed by globe-sparing surgery to resect the orbital neoplasm. Subsequently, she underwent adjuvant intravenous chemotherapy and was referred to the radiation oncologist for radiation of the orbit and the sites of bony metastasis. This case presentation highlights therapeutic advances in the management of adenoid cystic carcinoma of the lacrimal gland including the potential for targeted therapy based on the unique molecular basis of the tumor. 

Presentation Date: 09/12/2019

Issue Date: 08/01/2020