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. 2021 Feb;37(2):391-401.
doi: 10.1007/s00381-020-04833-x. Epub 2020 Jul 26.

Management of pediatric craniopharyngioma: 10-year experience from high-flow center

Abd El Rahman Enayet  1   2 , Mostafa M E Atteya  2   3 , Hala Taha  4 , Mohamed Saad Zaghloul  5 , Amal Refaat  6 , Eslam Maher  7 , Amal Abdelaziz  7 , Mohamed A El Beltagy  8   9
Affiliations

Management of pediatric craniopharyngioma: 10-year experience from high-flow center

Abd El Rahman Enayet et al. Childs Nerv Syst. 2021 Feb.

Abstract

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.

Keywords: Beta-catenin; Brain tumor; Conservative surgery; Endoscopy-assisted microsurgery; Pediatric craniopharyngioma.

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