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

AI Article Synopsis

  • Craniopharyngiomas are rare brain tumors in children, making up 1.2%-18.4% of cases, and while survival rates are decent, they bring long-term issues like recurrences and hormonal problems that affect overall quality of life.
  • A study tracking 12 pediatric patients over an average of 6.6 years found that common symptoms include raised intracranial pressure and vision problems, with survival at 75% but a high recurrence rate of 58%.
  • The research highlights the challenges in managing these tumors and suggests the need for more studies on improving treatment strategies and addressing the complications that arise from these conditions.

Article Abstract

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 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.

Methods: A 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.

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 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).

Conclusions: Long-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.

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Source
http://dx.doi.org/10.1016/j.wneu.2023.06.068DOI Listing

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