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Published on: October 20, 2017
Management of pediatric craniopharyngioma: 10-year experience from high-flow center
Abd El Rahman Enayet1,2, Mostafa M E Atteya2,3, Hala Taha4
1Neurosurgery Department, Kasr Al-Ainy School of Medicine, Cairo University, Cairo, Egypt.
Insights
This study reports on 137 childhood craniopharyngiomas, highlighting that multimodal management and conservative surgery balance quality of life with tumor control. Beta-catenin mutations correlate with aggressive behavior, informing individualized treatment strategies for pediatric craniopharyngioma.
Area of Science:
- Pediatric Oncology
- Neurosurgery
- Molecular Pathology
Background:
- Craniopharyngioma is a rare pediatric brain tumor.
- Effective management requires balancing tumor control with long-term quality of life.
Purpose of the Study:
- To review the 10-year experience with 137 childhood craniopharyngiomas.
- To analyze management strategies and outcomes.
- To assess the role of beta-catenin mutations.
Main Methods:
- Retrospective review of 137 pediatric craniopharyngioma cases (2007-2017).
- Analysis of surgical resection extent, radiotherapy, and adjuvant therapies.
- Immunohistochemical assessment of beta-catenin expression.
Main Results:
- Headache, visual failure, and hypothyroidism were common presenting symptoms.
- Gross total resection achieved in 35%, subtotal in 42%.
- Five-year progression-free survival was 52.3%; radiotherapy group showed better survival (72.25%) than follow-up (34.49%).
- Beta-catenin mutations (61/95 patients) trended towards aggressive behavior and lower PFS (39.4% vs 65.5%).
Conclusions:
- Conservative surgery and multimodal management are key for pediatric craniopharyngioma.
- Beta-catenin mutations may indicate a more aggressive clinical course.
- Individualized management protocols, like the CCHE-57357 algorithm, are essential.
Purpose:
To report our experience and management strategies during 10 years for 137 childhood craniopharyngiomas treated at a single institution.
Methods:
Medical records of children with craniopharyngioma treated at Children's Cancer Hospital Egypt (CCHE-57357) from July 2007 to December 2017 were retrospectively reviewed. Beta-catenin as an immunohistochemical marker was assessed also in available specimens.
Results:
Our registry included 137 patients. Headache (n = 122), visual failure (n = 118), and hypothyroidism(n = 78) were the most common findings on presentation. Three management protocols were identified; 65 patients were primarily followed up after surgery, 71 patients had radiotherapy after surgery, and one patient underwent surgery for Ommaya insertion with intracystic interferon injection. Overall, gross total resection/near total resection was achieved in 48 cases (35.04%), subtotal resection was achieved in 58 patients (42.33%), 29 (21.16%) had biopsy and Ommaya reservoir, and two patients with calcified lesions had no operations. Fifty-four patients showed recurrence/progression of their lesions. Allover, 5-year progression-free survival (PFS) was 52.3%, while it was 34.49% and 72.25% for the follow-up group and the radiotherapy group, respectively. Beta-catenin mutations were positive in 61/95 patients; 5-year PFS for beta-catenin negative and positive cases was 65.5% and 39.4% respectively (p = 0.087). Mortality was reported in eight patients. Intraoperative endoscopy-assisted assessment was the cornerstone of tailored decision-making.
Conclusion:
The concepts of conservative surgery and multimodal management should be applied to reach the perfect balance between the quality of life and the best tumor control rates. Beta-catenin mutations more than 5% are associated with statistically trending aggressive clinical behavior. The CCHE-57357 algorithm of individualized management protocol was presented.
