CP-06. PEDIATRIC CRANIOPHARYNGIOMA: SINGLE CENTER RETROSPECTIVE STUDY OF SURVIVAL, ENDOCRINOLOGIC, AND VISION OUTCOMES IN 90 PATIENTS

Abstract

Abstract BACKGROUND Craniopharyngioma is histologically benign. However, due to its location, morbidity is high and may be impacted by therapeutic approach. METHODS A retrospective study of patients treated for craniopharyngioma at Seattle Children’s Hospital from 1996-2023 was performed. Surgical approach, extent of resection, subsequent surgery, radiation and medical therapies, and serial surveillance magnetic resonance imaging for progression were reviewed. Five-year overall survival (OS) and progression free survival (PFS) were calculated and morbidity including endocrine and vision dysfunction was described. RESULTS 90 patients were included of which 53(58.9%) were male. Median age at diagnosis was 8.70 (range 1.39-25.17) years. Surgical approach included endoscopic (N=24), craniotomy (N=64), and combined (N=2). Extent of resection was biopsy N=2, subtotal resection N=7, near total resection N=38, gross total resection N=30. Pathology was adamantinomatous subtype in all cases and 68 had a documented CTNNB1 alteration and 3 had a BRAF alteration in those with molecular analysis. 5-year OS was 96.1% (SE 0.13) and 5-year PFS was 51.9% (SE0.21), with median follow-up of 97 months. Transcranial compared to endoscopic endonasal approach was associated with increased risk of progression (62% vs 30%, OR 1.23). 34 patients were treated with radiation upfront (N=19) or at progression (N=15). 55% of patients at diagnosis and an additional 30% in follow up had pituitary hormone dysfunction. Hypothalamic obesity was documented in 31% of patients and varied by surgical approach. At presentation, ophthalmologic abnormalities included optic nerve edema (18%) and optic nerve pallor (24%) and 30% had abnormal visual acuity. Following initial surgery, 50% had improvement in visual acuity, 35% stable and 15% had worsened visual acuity. CONCLUSIONS Upfront surgical strategy may impact risk of disease recurrence; however, its relationship to long term morbidity is not clear. Further work to delineate therapeutic strategies and their relationship to morbidity in this patient population is needed.