Primary malignant tumors of the sphenoidal sinus

OBJECTIVE: Neoplasms of the paranasal sinuses account for 0.2 to 0.8% of all cancers and for 2 to 3% of head and neck cancers. Primary involvement of the sphenoidal sinus has been reported to occur in only 1 to 2% of all paranasal sinus tumors. Tumors at this site commonly present with cranial neuropathies and require multidisciplinary treatment. METHODS: During a 21- year period, 27 patients with primary sphenoidal sinus tumors were evaluated. Those with pituitary tumors and fibro-osseous lesions (fibrous dysplasia) were excluded. The records of all remaining patients were reviewed for the following parameters: age, sex, and ethnic origin; presenting symptoms and signs; radiological features and sites of tumor extension; pathological findings; prior treatments; surgical approaches and extent of resection; adjuvant therapy; complications; and patient outcome. RESULTS: A malignant pathological process was found in 26 of the 27 patients. The most common diseases were squamous cell carcinoma (n = 9), adenoid cystic carcinoma (n = 4), chondrosarcoma (n = 3), and neuroendocrine carcinoma (n = 3). Treatments included surgery (n = 1), surgery and radiotherapy (n = 6), surgery and chemotherapy (n = 3), surgery, radiotherapy, and chemotherapy (n = 4), chemotherapy (n = 5), chemotherapy and radiotherapy (n = 3), and radiotherapy (n = 2). The mean follow-up period from initial evaluation was 41 months (range, 2-199 mo). At the last follow-up, 12 patients (48%) were still alive and 13 (52%) had died. The 2-year survival rate for patients with squamous cell carcinoma was 44%. CONCLUSION: Headache and visual disturbances are common symptoms of sphenoidal sinus tumors. The presence of cranial neuropathies, although common, suggests a less successful outcome. It is advantageous if the surgical team has had experience with both open and endoscopic approaches to the sphenoidal sinus. Patient outcome is maximized by the use of aggressive multidisciplinary therapy.