Neuro-ophthalmology of sphenoid sinus carcinoma

Three cases of sphenoid sinus carcinoma observed personally and 39 identified in the English language publications are reviewed. Sphenoid carcinoma constitutes only 0.3 per cent of sinus cancer. Its symptoms and signs are nonspecific until the sinus wall is penetrated. Once breached, specific neuro-ophthalmological symptoms and signs ensue, resulting from involvement of anatomically contiguous structures. These are characterized most commonly by the sphenocavernous syndrome and less frequently by isolated sixth nerve palsies and visual loss. Identified best by plain radiography, polytomography and computerized tomography, diagnosis of sphenoid carcinoma requires direct biopsy. Treatment, principally with radiotherapy supplemented by chemotherapy, has been disappointing with most patients dead by three years. The authors believe that in rapidly evolving neuro-ophthalmological syndromes of basal origin, accompanied by intractable headache, sphenoid sinus cancer must be considered.