Craniopharyngioma: An 18-Year Experience

Background Craniopharyngiomas are histologically benign but locally aggressive epithelial tumors of the sellar and suprasellar regions, which are often adherent to critical neurovascular structures. Herein, we describe our evolving surgical management of these complex tumors which continue to pose a formidable challenge for safe removal and long-term management.

Methods All patients undergoing craniopharyngioma resection by the main author over an 18-year period were reviewed. Demographics, tumor location and characteristics, surgical approach, adjuvant therapy, complications, visual and endocrine outcomes, symptom improvement, and tumor control rates were collected. Special attention was given to the evolution of operative approach and surgical technique, over the course of our experience.

Results From 2000 to 2017, a total of 58 patients (mean age: 44 years, 44% female) underwent 70 surgeries for craniopharyngioma resection; 32 (46%) of the surgeries were re-resections for recurrent or residual tumor. The surgical approaches included endonasal microscopic (6%), endonasal endoscopic-assisted (37%), fully endoscopic (37%), and supraorbital (20%). Since 2011, all patients undergoing a transsphenoidal resection utilized a fully endoscopic approach. Preoperative endocrinopathy was present in 79% of all cases. Postoperatively, one patient had a complete hormonal recovery, 36 (51%) patients had stable endocrinopathy, and 20 (29%) patients had worsened hormonal function. Of 15 patients without preoperative endocrinopathy, none experienced new postoperative hormonal dysfunction. Regarding vision, improvement was seen in 94% of 48 patients with preoperative visual dysfunction, greatest with first-time and fully endoscopic cases, while 2 patients had stable vision, and 1 experienced a postoperative homonymous hemianopia from a posterior cerebral artery spasm and subsequent stroke. Overall, 60% of patients had complete or near-complete resection, including 74% of first-time resections and 65% of all fully endoscopic cases. By surgical approach, complete/near-complete removal was accomplished in 63, 73, and 29% of microscopic and endoscope-assisted cases combined (n = 30), fully endoscopic cases (n = 26), and supraorbital cases (n = 16), respectively. Regarding skull base reconstruction and CSF leak repair in the endonasal cohort, postoperative CSF leaks were 14% in the microscopic and endoscope-assisted cohort (n = 30), and 8% in the fully endoscopic cohort (n = 26). Of the fully endoscopic cohort, 21 of 26 (81%) were repaired with a nasoseptal flap with one failure (5%) and one case of meningitis. Of 38 patients undergoing first-time surgery (20 fully endoscopic, 14 endoscope-assisted, 4 supraorbital), 50% received postoperative radiation (stereotactic radiotherapy n = 18 and SRS n = 1). Of these 19 patients, 3 (16%) required additional surgery for tumor control.

Conclusion Most craniopharyngiomas are optimally treated by the endonasal endoscopic approach. For recurrent craniopharyngiomas, the supraorbital route is a complimentary minimally invasive alternative that is ideal in many patients, providing excellent access, especially with endoscopic assistance. The goals of craniopharyngioma surgery should be an optimal functional outcome, focusing on visual recovery and hormonal functional preservation if possible. There remains a significant role for stereotactic radiotherapy for lasting tumor control, as well as emerging targeted medical therapies, all of which emphasize the need for an experienced multidisciplinary team.