We describe a 27-year-old female with a giant cell tumor of her sphenoid sinus, presenting with nasal obstruction and cranial neuropathies. Both the surgical and subsequent medical management are reviewed. Additionally, we review the overall presentation, pathophysiology, and management of giant cell tumors of the skull base. Current treatment recommendations are presented.A 27-year-old female presented to our institution with a 1-month history of retro-orbital headache, neck pain, nasal obstruction, and blurred vision. CT head identified a sphenoid mass with extension into the sella, clivus, and ethmoid sinuses ( Fig. 1). MRI outlined a clival mass extending into the sphenoid sinus and cavernous sinus with a mass effect on the contents of the sella turcica (Fig. 2). An endoscopic endonasal biopsy was performed with pathology consistent with GCTb (Fig. 3).The patient then presented to the emergency department 5 days later with new-onset diplopia of the right eye, nasal drainage, and worsening headache. She was found to have a right-sided cranial nerve VI palsy. MRI showed invasion into the cavernous sinus with encroachment on the optic chiasm and internal carotid arteries bilaterally.
ConclusionGiant cell tumors of the skull base are rare, benign, and locally aggressive, with high recurrence rates. The age of presentation is usually between 20 and 40 years with a predominance in females. The presenting clinical symptoms vary widely depending on tumor location. Hearing loss, headache, tinnitus, and dysfunction of the cranial nerves are the usual modes of presentation in skull base involvement. Surgical excision remains the treatment of choice, yet due to the possibility of incomplete resection and high recurrence rates, adjuvant therapy with denosumab may be necessary to improve local control. Our report, in combination with previous reports, provides evidence to support this as a potential new standard of care.