ESPE Abstracts

Introduction: Craniopharyngioma (CP) is a rare and highly recurrent tumor with no available nationwide data.

Objective: This retrospective study aims to characterize patients with CP treated at a national referral center.

Methods: Clinical records of patients aged 18 years or younger diagnosed with CP were analyzed. Data including clinical presentation, biochemical profiles, MRI findings, surgical interventions, and outcomes were collected. Descriptive statistical analysis was performed.

Results: Sixty-five patients aged 1 to 18 years were admitted with the diagnosis of CF between 1986 to 2022, with a mean follow-up time of 11.4 years (range 1.4-37.6). The mean age at diagnosis was 9.0 ± 4.7 years, with a slight female predominance (57%). Twelve of the subjects died as a result of the disease. Of 58 patients who underwent biopsy, the results showed adamantinomatous histology in 97% of cases. At diagnosis the most commonly reported symptoms were headache (80%), visual disturbances (57%), vomiting (22%), and polydipsia (15%). Over 50% exhibited pituitary dysfunctions at diagnosis. Somatotropic axis was affected in 32%, thyrotropic in 25%, corticotropic in 22%, gonadotropic in 17% and arginine-vasopresine (AVP) deficiency in 8%. Mean BMI was +1.06 SD at diagnosis and +2.3 SD on follow-up. Preoperative MRI was performed in 46 patients. Four patients had a Paris Scale = 0, 11 patients had a Paris Scale = 1 and 31 patients had a Paris Scale = 2. Mean tumor size was 25 ± 4.9 cm3. Surgical approach was transcranial in 54 (83%) and transsphenoidal in 8 (12%) patients. Ommaya implantation was performed in 29 (45%) cases, and 25 (39%) received adjuvant cranial radiotherapy. During the first 7 days after surgery 48% had AVP dysfunction. At follow-up after the first procedure, 55% developed thyrotropic, 49% corticotropic, 34% gonadal, 31% somatotropic axis deficits, and 35% persistent AVP dysfunction. Relapse occurred in 60% of patients. A mean time to first recurrence was 30 months. We did not find a significant association between Paris classification, tumor size and third ventricular involvement and tumor recurrence. Furthermore, there was no association between the age at diagnosis and number or time of recurrence.

Conclusion: This study provides insights into the clinical characteristics and treatment outcomes of pediatric CP patients in our country. Our data are similar to previously reported studies. CP poses significant risks of neuroendocrinologic sequelae and complications. Optimal patient care necessitates comprehensive data collection to understand disease progression and common complications, highlighting the importance of a multidisciplinary approach in managing these patients.