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


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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    ABSTRACT: Orbital apex syndrome is an uncommon disorder characterized by ophthalmoplegia, proptosis, ptosis, hypoesthesia of the forehead, and vision loss. It may be classified as part of a group of orbital apex disorders that includes superior orbital fissure syndrome and cavernous sinus syndrome. Superior orbital fissure syndrome presents similarly to orbital apex syndrome without optic nerve impairment. Cavernous sinus syndrome includes hypoesthesia of the cheek and lower eyelid in addition to the signs seen in orbital apex syndrome. While historically described separately, these three disorders share similar causes, diagnostic course, and management strategies. The purpose of this study was to report three cases of orbital apex disorders treated recently and to review the literature related to these conditions. Inflammatory and vascular disorders, neoplasm, infection, and trauma are potential causes of orbital apex disorders. Management is directed at the causative process. The cases described represent a rare but important group of conditions seen by the maxillofacial surgeon. A review of the clinical presentation, etiology, and management of these conditions may prompt timely recognition and treatment.
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