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Risk-adapted, long-term management in childhood-onset craniopharyngioma

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Abstract

Purpose

This report is a review of findings on the diagnosis, treatment, clinical course, follow-up, and prognosis of craniopharyngioma patients with special regard to clinical trials and long-term management.

Methods

Literature search on Pubmed for paper published after 1994.

Results

Craniopharyngiomas are rare, embryonic malformations of the sellar/parasellar region with low histological grade. Clinical manifestations are related to increased intracranial pressure, visual impairment, and hypothalamic/pituitary deficiencies. If the tumor is favorably localized, therapy of choice is complete resection, with care taken to preserve hypothalamic and optic functions. In patients with unfavorable tumor location (i.e. involvement of hypothalamic areas), recommended therapy is limited hypothalamus-sparing surgical strategy followed by irradiation. Irradiation has proven effective in treatment of recurrences and progression. Surgical lesions and/or anatomical involvement of posterior hypothalamic areas can result in serious sequelae, mainly hypothalamic syndrome.

Conclusions

It is crucial that craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients’ by experienced multidisciplinary teams in the context of multicenter trials.

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The author is supported by the German Childhood Cancer Foundation, Bonn, Germany.

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  1. Department of Pediatrics, Klinikum Oldenburg AöR, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133, Oldenburg, Germany

    Hermann L. Müller

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Informed consent was obtained from all individual participants included in KRANIOPHARYNGEOM 2000 (NCT00258453) and KRANIOPHARYNGEOM 2007 (NCT01272622).

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Müller, H.L. Risk-adapted, long-term management in childhood-onset craniopharyngioma. Pituitary 20, 267–281 (2017). https://doi.org/10.1007/s11102-016-0751-0

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