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

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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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Authors and Affiliations

  1. Neurosurgery Department, Kasr Al-Ainy School of Medicine, Cairo University, Cairo, Egypt

    Abd El Rahman Enayet & Mohamed A. El Beltagy

  2. Neurosurgery Department, Children’s Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt

    Abd El Rahman Enayet, Mostafa M. E. Atteya & Mohamed A. El Beltagy

  3. Neurosurgery Department, Faculty of Medicine, Helwan University, Cairo, Egypt

    Mostafa M. E. Atteya

  4. Clinical Pathology Department, Children’s Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt

    Hala Taha

  5. Radiation Oncology Department, Children’s Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt

    Mohamed Saad Zaghloul

  6. Diagnostic Radiology Department, Children’s Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt

    Amal Refaat

  7. Research Department, Children’s Cancer Hospital Egypt (CCHE, 57357), Cairo, Egypt

    Eslam Maher & Amal Abdelaziz

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  1. Abd El Rahman Enayet
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  2. Mostafa M. E. Atteya
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Correspondence to Mohamed A. El Beltagy.

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Enayet, A.E.R., Atteya, M.M.E., Taha, H. et al. Management of pediatric craniopharyngioma: 10-year experience from high-flow center. Childs Nerv Syst 37, 391–401 (2021). https://doi.org/10.1007/s00381-020-04833-x

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