• LimMervyn Jun RuiMJRDepartment of Neurosurgery, University Surgical Cluster, National University Health System, Singapore, Singapore; Saw Swee Hock School of Public Health, Singapore, Singapore. Electronic address: mervynlim@u.nus.edu., Rambert Guan Mou Wee, Natalie Min Yi Aw, Sherry Jiani Liu, Cindy Wei Li Ho, Kejia Teo, Sein Lwin, Tseng Tsai Yeo, Miriam Kimpo, and Vincent Diong Wen Nga.
• Department of Neurosurgery, University Surgical Cluster, National University Health System, Singapore, Singapore; Saw Swee Hock School of Public Health, Singapore, Singapore. Electronic address: mervynlim@u.nus.edu.
• World Neurosurg. 2023 Jul 4.

BackgroundCraniopharyngiomas arise from the Rathke pouch and account for 1.2%-18.4% of pediatric primary brain tumors. Despite relatively good survival outcomes, patients face long-term morbidity from recurrences, visual impairment, and endocrinopathies, which reduce quality of life. We examined the management of pediatric craniopharyngiomas, their recurrences, and subsequent neuroendocrine sequelae in a tertiary center in South-East Asia.MethodsA retrospective cohort of 12 paediatric patients (aged ≤18 years) with histologically confirmed diagnosis of craniopharyngioma treated from January 2002 to June 2017 was conducted. Data collected included demographics, clinical presentation, imaging data, treatment details, postoperative sequelae, and outcomes on mortality and recurrence. Survival analysis was conducted using Cox-proportional hazards model.ResultsThe median follow-up time was 6.60 years (1.9-11.5 years). The mean age was 7.6 years (standard deviation 4.8) and 7 patients (58.3%) were male. The most common presenting symptoms were raised intracranial pressure (7, 58.3%), visual deficits (6, 50.0%), and preoperative endocrine abnormalities (2, 16.7%). Five patients underwent gross total resection (41.7%), and 7 underwent subtotal resection (58.3%). Overall survival was 75.0% (9 patients), and recurrence was 58.0% (7 patients). Median time-to-recurrence was 5.87 months (0.23-33.7, interquartile range 15.8), and median progression-free survival was 4.16 years (0.18-10.1, interquartile range 5.29).ConclusionsLong-term management of pediatric craniopharyngioma remains difficult, with multiple recurrences and long-term neuroendocrine sequelae impairing quality of life for patients. Further research into management of recurrences and neuroendocrine sequelae, as well as novel therapies to improve outcomes in these patients, may be warranted.Copyright © 2023 Elsevier Inc. All rights reserved.

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