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Craniopharyngioma in children. An age-based analysis on our 11 year experience with the endoscopic endonasal approach
Mirko Scagnet1, Rina Agushi2, Federico Mussa1
1Department of Pediatric Neurosurgery, Meyer Children's Hospital IRCCS, Viale Gaetano Pieraccini 24, 50139, Florence, Italy.
Insights
Craniopharyngiomas in children show age-dependent differences. Younger children (<6 years) present with larger tumors and visual issues, while teenagers experience diagnostic delays. Endoscopic endonasal approach (EEA) is effective across all pediatric age groups.
Area of Science:
- Pediatric Neurosurgery
- Endoscopic Skull Base Surgery
- Pediatric Oncology
Background:
- Craniopharyngiomas are rare pediatric brain tumors affecting the pituitary region.
- Management strategies require age-specific considerations due to varying tumor characteristics and clinical presentations.
- The endoscopic endonasal approach (EEA) offers a minimally invasive option for tumor resection.
Purpose of the Study:
- To conduct an age-based analysis of pediatric craniopharyngiomas treated with EEA.
- To define optimal clinical and surgical management criteria for different pediatric age groups.
- To evaluate the efficacy and outcomes of EEA in pediatric craniopharyngioma treatment.
Main Methods:
- Retrospective study of 29 pediatric patients treated with EEA between 2013 and 2024.
- Patients were stratified into three age groups: 3-6 years (Group A), 7-12 years (Group B), and 13-18 years (Group C).
- Comprehensive pre-operative evaluations (endocrinological, ophthalmological, radiological) and post-operative follow-up were performed.
Main Results:
- Younger children (Group A) exhibited more aggressive, voluminous tumors with higher recurrence rates and obesity risk.
- Visual impairment was more frequent in Group A (3-6 years) compared to Group C (13-18 years) (p=0.02).
- Diagnostic delays were significantly longer in teenagers (Group C) compared to pre-pubertal children (Group B) (23.4 vs. 7.7 months).
Conclusions:
- Age-based analysis reveals distinct craniopharyngioma characteristics and management challenges in pediatric patients.
- Younger children (<6 years) present with larger tumors and visual deficits that may not improve post-surgery.
- EEA is a feasible and effective approach for craniopharyngioma resection across all pediatric age groups, with higher gross total resection rates when used as primary treatment.
Objective:
The aim of this work is to perform an age-based analysis to define the criteria for the best management from a clinical and surgical point of view of craniopharyngiomas in children on patients treated with an endoscopic endonasal approach.
Methods:
For this retrospective study, the databank of Meyer Children's Hospital IRCCS was used. 29 consecutive patients treated with an endonasal endoscopic technique between January 2013 and January 2024 were enrolled in the study. All patients underwent endocrinological, ophthalmological and radiological examination upon admission. Standard follow-up appointments at the outpatient clinic were given to the patients at 1, 3 months and 6 months postoperatively and successively annual appointments were made.
Results:
The mean follow-up was 5.6 5.6 years ± 4.1 years. 40% of the patients presented with hydrocephalus, 41% of the children had an endocrine dysfunction as the primary symptom and visual impairment was present in 55% of the cases. Patients were subdivided into 3 groups according to age sub-stratification. Group A included young patients with ages between 3-6 years old (9 patients), Group B consisted of children in the pre-puberal age (7-12 years old - 12 patients) and Group C was formed by teenagers (13-18 years old - 8 patients). Group A presented more aggressive and voluminous tumors, with a higher recurrence and a higher risk of obesity. The period from first symptoms to diagnosis was significantly higher in Group C compared to Group B: 23.4 months versus 7.7 months respectively. Visual impairment was more frequent in Group A compared to Group C (p = 0.02).
Conclusions:
The age-based analysis showed some interesting findings. Younger children with an age inferior to 6 years old tend to present with more voluminous tumors and visual impairment as the first symptom, Once visual symptoms are manifested, they tend to be unchanged and do not show improvement, even after surgery. In teenagers the diagnosis can be delayed due to the misrecognition of the symptoms. However, our results showed that EEA is useful and feasible in all age groups, achieving a gross total/subtotal resection even in cases of recurrence. If used as the first treatment of choice the chances of gross total resection without further recurrence are higher.
