2026 Poster Presentations
P277: SURGICAL OUTCOMES IN PAPILLARY CRANIOPHARYNGIOMAS FOLLOWING ENDOSCOPIC ENDONASAL APPROACH
Sharath Kumar Anand, MD; Brock Gjesdal; Robert Dambrino, MD; Anthony Tang; Akanksha Aggarwal, MD; Katherine Mastandrea, MD; Divya Sistla, MD; Pouneh Fazeli, MD; Garrett Choby, MD; Carl Snyderman, MD, MBA; Georgios Zenonos, MD; Paul Gardner, MD; UPMC
Introduction: Recent studies have established that BRAF V600E-positive papillary craniopharyngiomas represent a distinct clinical and pathological entity, with unique radiographic features and a predilection for adult patients. The emergence of effective BRAF/MEK inhibitor therapy, demonstrated to induce dramatic tumor shrinkage and high response rates in both neoadjuvant and adjuvant settings, has broadened the treatment paradigm and raised important questions about the relative risks and benefits of surgery versus targeted therapy for this patient population. This study aims to describe our experience in the endoscopic endonasal management of BRAF V600E-positive craniopharyngioma patients.
Methods: This is a single-institution retrospective cohort study of consecutive patients who underwent endoscopic endonasal approach (EEA) for resection of craniopharyngioma from 2008 to 2024. Pathology reports were reviewed to identify those patients with papillary craniopharyngiomas and molecular studies reviewed to identify those specifically with BRAF-V600E positive mutations. Outcome measures included visual improvement, new diabetes insipidus (DI), new panhypopituitarism, complications, and recurrence/progression.
Results: We identified 17 patients with papillary craniopharyngiomas, 11 of whom were confirmed to have BRAF-V600E positive mutations while the remaining 6 still have pending molecular studies. Of these patients, 10 (58.8%) were male, average age was 57.6±14.9 years, 13 (76.5%) had a preoperative visual acuity or field deficit. 8 (47.1%) had gross total resection (GTR) and 8 (47.1%) had near-total resection. 4 patients had no visual deficits before and after surgery, while 13 patients presented with preoperative visual deficits. All patients had positive visual outcomes: 8 (61.5%) had partial improvement, and 5 (38.5%) had complete normalization. 11 (64.7%) patients developed new permanent DI. While 7 (41.2%) patients progressed to panhypopituitarism, the only 2 patients with normal preoperative pituitary function developed partial but not panhypopituitarism. Postoperative cerebrospinal fluid leak occurred in 2 (11.8%) patients. With median follow-up of 26 months (IQR 14-47), 5 (29.4%) patients demonstrated recurrence/progression: 2 patients were managed with gamma knife radiosurgery, 1 with repeat EEA, 2 were lost to follow-up.
Conclusions: Patients who underwent EEA for BRAF-V600E positive mutation craniopharyngiomas demonstrated glaring improvements in preoperative visual deficits but also experienced significant postoperative endocrinologic morbidity. While the effect of targeted immunotherapy for papillary craniopharyngiomas on visual deficits and endocrinologic outcome has yet to be described, the role for aggressive initial surgical management for these patients, especially with visual deficits, should continue to be considered and assessed.