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Omar Ozgur, Kateki Vinod, Peter Chang, Payal Patel, Lisabeth Hall, Elizabeth Maher; Diplopia as a Presenting Symptom of Adenoid Cystic Carcinoma of the Head and Neck. Invest. Ophthalmol. Vis. Sci. 2014;55(13):4095.
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To report 2 cases of adenoid cystic carcinoma (ACC) of the head and neck manifesting as multiple cranial nerve (CN) palsies causing diplopia as the presenting symptom, and to propose pathophysiologic mechanisms for this atypical presentation.
Two patients with ACC presenting with binocular diplopia were identified. Patient 1 (P1) was a 49-year-old man with vertical diplopia for 2 months, and right (R) forehead numbness for 2 weeks. Vision was 20/20 OU, and abduction and depression deficit OD were consistent with R CN IV palsy, with hypoesthesia in the R CN V1 distribution. Patient 2 (P2) was a 49-year-old woman with horizontal diplopia for 1 day. Vision was 20/30 OD and 20/25 OS. R facial hypoesthesia, abduction deficit OD, and R facial weakness were consistent with CN V, VI, and VII palsies. She soon developed R CN IV palsy, followed by R CN III palsy.
P1’s MRI showed R superolateral orbital inflammation with normal lacrimal gland (LG) without localizable mass. After a failed trial of oral steroids for presumed orbital pseudotumor, repeat MRI showed probable extension into the cavernous sinus (CS). R orbital biopsy showed orbital ACC from ectopic LG tissue. P2’s MRI showed an enhancing mass in the R CS. Endonasal biopsy demonstrated ACC. Fine needle aspiration biopsy confirmed the R submandibular gland as the primary source. Both patients were referred to oncology.
ACC is a rare malignant epithelial tumor that, in the head and neck, most commonly arises in the salivary glands, and less often, the LG. We describe 2 cases of ACC presenting atypically with diplopia. In P1, invasion of the superior orbital fissure likely accounts for CN IV involvement, resulting in vertical diplopia. In P2, perineural spread by ACC into the CS affecting CN VI resulted in horizontal diplopia. Review of the literature confirms that diplopia as a presenting symptom of ACC is very rare. To our knowledge, only 8 reports exist of ACC presenting with diplopia, due either to mass effect, perineural invasion, or CS involvement.
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