Diagnostics, treatment, and follow-up in craniopharyngioma.

Hermann L Müller

doi:10.3389/fendo.2011.00070

Abstract

Craniopharyngiomas are partly cystic embryogenic malformations of the sellar and parasellar region, with up to half the 0.5–2.0 new cases per million population per year occur in children and adolescents. Diagnosis profile for pediatric and adult craniopharyngioma is characterized by a combination of headache, visual impairment, and polyuria/polydipsia, which can also include significant weight gain. In children, growth retardation, and/or premature puberty often occur later or postoperatively. Recommended therapy with favorable tumor localization is complete resection; with unfavorable tumor localization (optic nerve and/or hypothalamic involvement), consensus is still pending whether a limited resection followed by local irradiation is more prudent. Even though overall survival rates are high (92%), recurrences after complete resection and progressions after incomplete resection can be expected. Accordingly, a randomized multinational trial (KRANIOPHARYNGEOM 2007) has been established to identify optimal diagnosis, treatment (particularly the ideal time point of irradiation after incomplete resection), and quality of life strategies of this chronic disease – most notably the morbid hypothalamic obesity in ∼50% of long-term survivors. We report on craniopharyngioma origins, its pathological manifestations, and specific challenges these sequelae pose regarding diagnosis, treatment, and life-long multi-discipline quality of life management for both adult and childhood craniopharyngioma patients.

Highlights

Craniopharyngioma is a rare, non-glial, non-malignant intracranial tumor derived from a malformation of embryonal tissue
We report on craniopharyngioma origins, its pathological manifestations, and specific challenges these sequelae pose regarding diagnosis, treatment, and life-long multi-discipline quality of life management for both adult and childhood craniopharyngioma patients
A retrospective study of functional capacity using the FMH instrument that quantifies patients’ abilities to perform everyday psychomotor tasks (Wolff et al, 1996) found significantly lower rankings for craniopharyngioma patients compared to age-matched controls (Müller et al, 2003a, 2004b), with craniopharyngioma patients’ morbid hypothalamic obesity cited as responsible for their low functional capacity self-rating

Summary

Introduction

Craniopharyngioma is a rare, non-glial, non-malignant intracranial tumor derived from a malformation of embryonal tissue. The most recent of the three reports (Müller et al, 2011b) analyzed craniopharyngioma outcome and prognosis relative to patient load of the treating neurosurgical centers, finding centers with lower craniopharyngioma patient load – and relatively less experience treating craniopharyngioma – tended to use more radical surgical approaches, resulting in less favorable outcomes regarding obesity and other QoL considerations.

Results

Conclusion