Long-term endocrine complications and quality of life in pediatric-onset craniopharyngioma: a single-center cohort study
摘要
Craniopharyngioma is a benign yet locally aggressive brain tumor that causes substantial long-term endocrine, metabolic, visual, and hypothalamic morbidities, leading to impaired quality of life (QoL). However, data on QoL and long-term outcomes in Thai pediatric patients remain limited.
MethodsThis study included two components: a retrospective cohort review of long-term endocrine, metabolic, and visual outcomes in patients aged ≤ 25 years with histologically confirmed pediatric-onset craniopharyngioma diagnosed between 2000 and 2023 at Siriraj Hospital, and a cross-sectional assessment of quality of life using the Pediatric Quality of Life Inventory (PedsQL 4.0). Factors associated with quality of life were evaluated using linear regression.
ResultsEighteen patients were included (median age at diagnosis 6.8 years). Postoperative endocrine deficiencies were common, including growth hormone deficiency (88.9%), vasopressin deficiency (100%) and gonadotropin deficiency (93.3% of evaluable patients). Residual/recurrent disease developed in 55.6% of patients during follow-up. Metabolic complications were common, overweight and obesity at recent follow-up were observed in 6 (33.3%) and 4 (22.2%) patients, respectively. Among screened patients (88.9%), dyslipidemia occurred in 62.5%, metabolic dysfunction–associated steatotic liver disease in 25.0%, and type 2 diabetes in one patient. Height SDS declined and BMI SDS increased significantly over time (p < 0.05). Visual impairment progressed in 27.8%. The mean PedsQL score was 68.8 ± 15.2. In exploratory analyses, obesity was associated with poorer quality of life.
ConclusionsPediatric craniopharyngioma carries a substantial burden of long-term endocrine, metabolic, and visual morbidity, adversely affecting growth and quality of life. Lifelong, multidisciplinary follow-up is essential to optimize outcomes.