Primary sphenoid sinus neoplasms: a report of four cases with common clinical presentation treated with transsphenoidal surgery and adjuvant therapies

Background: Primary neoplasms of the sphenoid sinus are a rare occurrence, accounting for approximately 1-2% of all paranasal sinus tumors. Here we report a series of four such patients. Methods: Four patients with sphenoid sinus neoplasms were identified (1%), all treated during the year 2003. Results: The four patients included two women and two men (mean age 61 years, range 44-70 years). Two patients presented with unilateral abducens cranial nerve (CN) palsies; one had trigeminal facial numbness and dizziness; another had headache, epistaxis, and partial third and fourth CN palsies. MRIs in all patients demonstrated large sphenoid sinus masses with partial clival and sellar bone erosion but with clear visualization of the pituitary gland above the mass. Cavernous sinus invasion was present in all four cases, including one patient with tumor in the ethmoid sinus and intra-tumoral hemorrhage. No patients had endocrinopathy. All patients underwent subtotal tumor removal via an endonasal transsphenoidal route. Tumor histology included neuroendocrine carcinoma, sinonasal undifferentiated carcinoma, mucoepidermoid carcinoma, and giant cell tumor. Post-operatively, all four patients had improved symptoms with complete resolution of diplopia in 3 of 3 patients. Metastatic work-ups were negative in all patients, and all received fractionated stereotactic radiotherapy; three received chemotherapy. One patient required a second endonasal tumor debulking 15 months after the first for new visual loss that then resolved. At a median follow-up of 21 months, all patients are alive and functional. Conclusion: Intra-sphenoidal tumors are locally invasive tumors that include a wide pathological spectrum. In this small series, they presented with cavernous sinus symptoms and headache but not endocrinopathy. Recognizing their distinctive clinical presentation and MRI features is helpful in differentiating them from primary sellar tumors. Their aggressive nature warrants a multimodality treatment plan including surgical debulking, radiotherapy, and chemotherapy in some cases.