Childhood craniopharyngioma: treatment strategies and outcomes

Abstract

Craniopharyngiomas are embryogenic malformations of the sellar region. With an overall incidence of 0.5–2.0 new cases per million population per year, approximately 30–50% of all cases represent childhood craniopharyngioma. Therapy of choice in patients with favorable tumor localization is complete resection with a specific focus on maintaining functions of the optical nerve and hypothalamic-pituitary axes. In patients whose unfavorable tumor localization makes maintaining hypothalamic functionality surgically challenging, a limited resection followed by local irradiation is recommended. The overall survival rates are high (92%) but relapses and reduced QoL in survivors are also frequent. Because irradiation is efficient in preventing tumor progression, appropriate timing of post-surgical irradiation is currently under investigation in a randomized trial (KRANIOPHARYNGEOM 2007). Childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the clinical and QoL consequences in order to provide optimal care of surviving patients.

Keywords::

• brain tumors
• craniopharyngioma
• hypothalamic obesity
• irradiation
• neurocognitive
• neurosurgery
• pituitary
• quality of life
• recurrence

Acknowledgements

The author is grateful for the help of M Neff-Heinrich (Göttingen, Germany) in proofreading and editing the manuscript.

Financial & competing interests disclosure

The author is supported by a grant from the German Childhood Cancer Foundation. The author has no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• For favorably localized craniopharyngiomas, the preferred treatment of choice is an attempt at complete resection with preservation of visual, hypothalamic and pituitary function.

• For unfavorably localized tumors with close proximity to optical and/or hypothalamic structures, a radical neurosurgical strategy attempting complete resection is not recommended in order to prevent severe sequelae.

• Hypothalamic tumor involvement and neurosurgical hypothalamic lesions have a major negative impact on quality of life in survivors, mainly due to hypothalamic obesity.

• Irradiation is effective in preventing recurrences and progressions.

• The appropriate time point of irradiation in patients with residual postoperative tumor is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007).

• Currently, no generally accepted (pharmacological or bariatric) therapy for hypothalamic obesity in craniopharyngioma has been shown to be effective in randomized studies.

• Childhood craniopharyngioma should be recognized as a chronic disease requiring constant monitoring of the consequences and medical resources for treatment by experienced multidisciplinary teams.

Notes