Management and Outcomes of Pediatric Craniopharyngioma: A 15-Year Experience in Singapore

-BACKGROUND: Craniopharyngiomas 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 re-currences, visual impairment, and endocrinopathies, which reduce quality of life. We examined the management of pediatric craniopharyngiomas, their recurrences, and sub-sequent neuroendocrine sequelae in a tertiary center in South-East Asia. -METHODS: A retrospective cohort of 12 paediatric pa-tients (aged 18 pound years) with histologically confirmed diagnosis of craniopharyngioma treated from January 2002 to June 2017 was conducted. Data collected included de-mographics, clinical presentation, imaging data, treatment details, postoperative sequelae, and outcomes on mortality and recurrence. Survival analysis was conducted using Cox-proportional hazards model. -RESULTS: The 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 preop-erative endocrine abnormalities (2, 16.7%). Five patients -nderwent 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, inter-quartile range 15.8), and median progression-free survival was 4.16 years (0.18-10.1, interquartile range 5.29). CONCLUSIONS: Long-term management of pediatric craniopharyngioma remains difficult, with multiple re- currences and long-term neuroendocrine sequelae impairing quality of life for patients. Further research into management of recurrences and neuroendocrine sequelae, well as novel therapies to improve outcomes in these patients, may be warranted.

Automated summary

The long-term management of pediatric craniopharyngioma remains challenging, with multiple recurrences and long-term neuroendocrine sequelae affecting the quality of life for patients. Further research into the management of recurrences and neuroendocrine sequelae, as well as novel therapies to improve outcomes, may be necessary.