Primary Adenoid Cystic Carcinoma of the Orbital Apex

Departments of *Ophthalmology, Scheie Eye Institute, †Radiation Oncology, ‡Otorhinolaryngology-Head and Neck Surgery, and §Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, U.S.A.
Ophthalmic plastic and reconstructive surgery (Impact Factor: 0.88). 10/2012; 29(1). DOI: 10.1097/IOP.0b013e31826463be
Source: PubMed

ABSTRACT Adenoid cystic carcinoma is an uncommon malignant tumor of epithelial origin typically arising from salivary glands. Orbital involvement may occur via direct or perineural spread from a lacrimal gland or sinonasal source. Primary orbital adenoid cystic carcinoma without involvement of the lacrimal gland is rare. The authors report a 53-year-old woman who was examined for insidious monocular vision loss and was found to have a primary adenoid cystic carcinoma of the orbital apex and cavernous sinus. Systemic workup for a primary source, including ipsilateral lacrimal gland biopsy, was negative. One must maintain a high index of suspicion for adenoid cystic carcinoma when evaluating orbital tumors.

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