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Published in: Child's Nervous System 2/2021

01-02-2021 | Craniopharyngioma | Original Article

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

Authors: Abd El Rahman Enayet, Mostafa M. E. Atteya, Hala Taha, Mohamed Saad Zaghloul, Amal Refaat, Eslam Maher, Amal Abdelaziz, Mohamed A. El Beltagy

Published in: Child's Nervous System | Issue 2/2021

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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.
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Metadata
Title
Management of pediatric craniopharyngioma: 10-year experience from high-flow center
Authors
Abd El Rahman Enayet
Mostafa M. E. Atteya
Hala Taha
Mohamed Saad Zaghloul
Amal Refaat
Eslam Maher
Amal Abdelaziz
Mohamed A. El Beltagy
Publication date
01-02-2021
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 2/2021
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-020-04833-x

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