ESPE Abstracts

Introduction: Childhood onset craniopharyngioma (CP) is a rare, low-grade (WHO I) embryonic malformation arising from remnants of the craniopharyngeal duct in the sellar and parasellar regions.

Methods: This retrospective study included 167 children (53% boys, 47% girls) diagnosed with CP at the CMHI, Warsaw, Poland, from 1998 to 2024. We evaluated anthropometric data, hormonal test results pre- and post-surgery, brain magnetic resonance imaging (MRI) findings, and histopathological and immunohistochemical outcomes.

Results: The median age at diagnosis was 9.7 years (IQR: 6.4-12.6). The median interval from symptom onset to diagnosis was 1.4 years (IQR: 0.28-3.48). The most frequent symptoms included ophthalmological complications (66,5%), headaches (65.9%), and growth deceleration (51.8%). Symptoms prompting further diagnostics were primarily headaches (42%), vomiting (22%), ophthalmologic complications (19%), and short stature (18%). Hypothalamic-pituitary axis assessments were conducted preoperatively in 77.9% of patients. Growth hormone deficiency (GHD) was found in 90.1% (40/44), secondary hypothyroidism in 37.2% (48/129), secondary adrenal insufficiency in 29.2% (31/106), hypogonadotropic hypogonadism in 66.7% (16/24), and arginine vasopressin deficiency (AVD) in 10.8% (18/166). The median tumor diameter on MRI was 35 mm (IQR: 28-45.4), with a range of 10-100 mm. According to Puget’s grading system, tumors were classified as grade 0 (8.4%), grade 1 (26.6%), and grade 2 (62%). All patients were diagnosed with adamantinomatous craniopharyngioma. Total tumor resection was achieved in 75.5% of patients, 28.6% received adjuvant radiotherapy. Postoperatively, GHD was identified in 66.9% (109/163), secondary hypothyroidism in 97% (161/166), hypogonadotropic hypogonadism in 52.7% (87/165), AVD in 92.8% (154/166), and partial AVD in one patient. Syndrome of inappropriate antidiuretic hormone (SIADH) occurred in 47.7% (31/65). 97% of patients required hydrocortisone (HCT) postoperatively; 6,2% of patients achieved hydrocortisone supplementation cessation. There were ten deaths, with a median OS of 1.57 years (IQR: 0.8-2.9). Two-year, five-year, and ten-year survival rates were 96%, 94%, and 92%, respectively.

Conclusion: Growth deceleration was a common and early symptom, suggesting that regular monitoring of anthropometric data in children could facilitate earlier CP diagnosis. At the diagnosis, AVD occurring in 10.8% of cases, was less common compared to germinoma. Postoperative SIADH was frequently observed. In some patients postoperatively diagnosed with secondary adrenal insufficiency, the cortisol level normalizes in follow-up.