Cavernous Sinus Syndrome Resulting from Contiguous Spread of Adenoid Cystic Carcinoma: A Systematic Analysis of Reported Cases

Purpose: The aim of this review is to analyze all reported cases of histologically confirmed adenoid cystic carcinoma (ACC) with cavernous sinus involvement identified in the literature in order to elucidate the clinical symptoms, neuro-ophthalmic and neuro-imaging findings. Methods: A systematic electronic database search (Medline, Biosis and Cochrane) and a literature review were used to identify reported cases which were assessed for demographic information; primary tumour site; pattern of spread; extent and timing of cranial nerve involvement; and neuro-imaging findings. Conclusions: Neuro-ophthalmologic evaluation is extremely important in the diagnosis of this disease which has a propensity for perineural and direct orbital invasion.