Akademik Veri Yönetim Sistemi
Journal of clinical research in pediatric endocrinology, cilt.16, sa.3, ss.288-296, 2024 (SCI-Expanded)
Objective: Craniopharyngiomas (CPG) have complex treatment challenges due to their proximity to vital structures, surgical and
radiotherapeutic complexities, and the tendency for recurrence. The aim of this study was to identify the prevalence of endocrine and
metabolic comorbidities observed during initial diagnosis and long-term follow-up in a nationwide cohort of pediatric CPG patients. A
further aim was to highlight the difficulties associated with CPG management.
Methods: Sixteen centers entered CPG patients into the ÇEDD NET data system. The clinical and laboratory characteristics at presentation,
administered treatments, accompanying endocrine, metabolic, and other system involvements, and the patient’s follow-up features were
evaluated.
Results: Of the 152 evaluated patients, 64 (42.1%) were female. At presentation, the mean age was 9.1±3.67, ranging from 1.46 to
16.92, years. The most common complaints at presentation were headache (68.4%), vision problems (42%), short stature (15%), and
nausea and vomiting (7%). The surgical procedures were gross total resection (GTR) in 97 (63.8%) and subtotal resection in 55 (36.2%).
Radiotherapy (RT) was initiated in 11.8% of the patients. Histopathological examination reported 92% were adamantinamatous type and
8% were papillary type. Postoperatively, hormone abnormalities consisted of thyroid-stimulating hormone (92.1%), adrenocorticotropic
hormone (81%), antidiuretic hormone (79%), growth hormone (65.1%), and gonadotropin (43.4%) deficiencies. Recombinant growth
hormone treatment (rhGH) was initiated in 27 (17.8%). The study showed hesitancy among physicians regarding rhGH. The median
survival without relapse was 2.2 years. Median (range) time of relapse was 1.82 (0.13-10.35) years. Relapse was related to longer followups and reduced GTR rates. The median follow-up time was 3.13 years. Among the last follow-up visits, the prevalence of obesity was
38%, but of these, 46.5% were already obese at diagnosis. However, 20% who were not obese at baseline became obese on follow-up.
Permanent visual impairment was observed in 26 (17.1%), neurological deficits in 13 (8.5%) and diabetes mellitus in 5 (3.3%) patients.
Conclusion: Recurrence was predominantly due to incomplete resection and the low rate of postoperative RT. Challenges emerged for
multidisciplinary regular follow ups. It is suggested that early interventions, such as dietary restrictions and increased exercise to prevent
obesity, be implemented.