Case Report – Resection of Rare Clival Giant Cell Tumor by Le Fort I Osteotomy Approach

Introduction: Giant cell tumor (GCT) is a rare bone tumor, which comprises 5% of all skeletal tumors. GCTs involving the skull tend to arise in the sphenoid or petrous part of the temporal bone and account for less than 1% of all bone GCTs. The typical patient profile for GCT includes young Asian females in the second to third decades of life, Because GCTs in the skull are extremely rare, there is little established about the appropriate management and therapy. Reported cases of clival GCTs use a transsphenoidal or transmaxillary surgical approach. We describe a rare case of a GCT originating in the clivus in a middle aged white male with a Le Fort I osteotomy and median maxillotomy surgical approach.

Case Presentation: A 49-year old male (DM) presented to the emergency department in June 2013 with complaints of headaches and blurred vision in the right eye for the past 2 weeks. Contrast CT scans revealed a 4.9 × 3.2 cm suprasellar mass with erosion through the sphenoid sinuses and compression of the optic chiasm and right-sided optic nerve. Transsphenoidal biopsy was consistent with giant cell tumor.

Management and Outcome: Patient DM underwent a definitive resection of the clival GCT in August 2013. Patient underwent an initial tracheostomy followed by a transoral approach to expose the anterior skull base by Le Fort I Osteotomy and median maxillotomy with palatal split. Following the exposure of the sella turcica and inferior clivus, micro-dissection and surgical cavity exploration was performed using the field microscope and stereotactic navigation. Following surgery, patient was monitored in the hospital for 12 days where he was weaned off the ventilator. During the hospital course and upon discharge, patient DM showed no neurological deficits, intact cranial nerves and no visual defects.

Discussion: Giant cell tumors of clival origin are extremely rare and patient DM's case contributes to the limited amount of reported skull-based GCT cases worldwide. In addition, the Le Fort I Osteotomy approach, an established surgical approach for skull-based lesions, has never been performed for clival GCTs to our knowledge. The surgery was successful with no complications confirming that the Le Fort I and median maxillotomy method is appropriate for clival tumors.