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S182: POSTOPERATIVE OUTCOMES FOR 28 PRIMARY AND RECURRENT CRANIOPHARYNGIOMAS IN CHILDREN
Spencer M Frome, MA; Yosef Dastagirzada, MD; Jeffrey Wisoff, MD; Department of Neurosurgery, Division of Pediatric Neurosurgery, NYU Langone Medical Center, New York, NY

Introduction: Craniopharyngiomas are tumors of the sella that account for 6 – 9% of all pediatric brain tumors. Despite their benign pathology, they often cause significant morbidity due to hypothalamic dysfunction and optic interference. Treatment options typically include gross total resection (GTR) or subtotal resection (STR) followed with radiation; however, the optimal long-term approach remains debated.  

Objective: To report the demographics and outcomes of resection for previously unpublished pediatric craniopharyngioma patients at our institution.

Methods: We conducted a retrospective analysis of 28 pediatric patients with pathologically confirmed adamantinomatous craniopharyngiomas, treated surgically between January 2007 and August 2024.

Results: The mean age of presentation was 7.3 for primary craniopharyngiomas in comparison to 12.3 for the recurrent cohort (p = 0.009). Of 28 pediatric patients, 10 (36%) had prior treatments at other institutions, including radiation (7), ommaya placement (6), STR (5), cyst drainage (4), GTR (1), or chemotherapy (1). No other significant demographic or tumor characteristic differences were observed between these groups. Five patients (18%) presented with hydrocephalus, and 10 (36%) were neurologically intact on presentation. There were 16 (57%) retrochiasmatic, 9 (32%) prechiasmatic and 3 (11%) complex craniopharyngiomas with a mean size of 3.4 cm (SD 1.5) in maximum diameter. Mean follow up was 109 months (SD 56). Twenty-five (89%) of the resections were performed via a frontal-orbital-zygomatical-temporal approach.

In the patients who received their first treatment at our institution, 17/18 (94%) resections achieved GTRs compared to only 3/10 (30%) in the recurrent group (p = <0.001). Only 3 patients across the cohort experienced improved pre-operative neurological deficit, all of whom had not received previous treatments. Notably, 4/10 (40%) patients in the recurrent group experienced a new post-operative deficit, compared to only 1/18 (6%) in the first presentation group (0 = 0.041). There were no significant endocrine differences across Puget scores, likely due to small sample sizes. All patients receiving GTR (20) experienced DI and required a mean of 4.0 (±0.9) pituitary replacement medications, versus 5/8 (63%) patients with DI and a mean of 2.5 (±1.7) medications with STR (p values 0.017 and 0.043, respectively).

Kaplan-Meier curves for recurrence-free survival (RFS) indicated that patients receiving GTR or treated first at our institution had better RFS outcomes (Log-rank p <0.001 and 0.024, respectively). Of patients with adequate follow-up, 23/26 (88%) achieved stable disease control.

Conclusions: These results complement findings from our previously published cohort of 86 pediatric craniopharyngioma cases. Maximum safe resection is effective for disease control in pediatric patients. Achieving GTR significantly increases the likelihood of a patient experiencing recurrence free survival, though it is associated with a higher pituitary replacement burden. Patients treated after prior interventions often face more complex courses with higher recurrence and neurological deficit rates.

 

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