Childhood Craniopharyngioma – Changes of Treatment Strategies in the Trials KRANIOPHARYNGEOM 2000/2007

Abstract

Background: Prognosis in childhood cranio­pharyngioma, is frequently impaired due to sequelae. Radical surgery was the treatment of choice for decades. Even at experienced facilities radical surgery can result in hypothalamic disorders such as severe obesity.

Objective: We analyzed, whether treatment strategies for childhood craniopharyngioma patients recruited in GPOH studies have changed during the last 12 years.

Materials and methods: We compared the grade of pre-surgical hypothalamic involvement, treatment, degree of resection and grade of surgical hypothalamic lesions between patients recruited in KRANIOPHARYNGEOM 2000 (n=120; 2001–2007) and KRANIOPHARYNGEOM 2007 (n=106; 2007–2012).

Results: The grade of initial hypothalamic involvement was similar in patients treated 2001–2007 and 2007–2012. The realized treatment was more radical (p=0.01) in patients recruited 2001–2007 (38%) when compared with patients treated 2007–2012 (18%). In patients with pre-surgical involvement of anterior/posterior hypothalamic areas, the rate of hypothalamus-sparing operations resulting in no (further) hypothalamic lesions was higher (p=0.005) in patients treated 2007–2012 (35%) in comparison with the 2001–2007 cohort (13%). Event-free-survival rates were similar in both cohorts.

Conclusions: A trend towards less radical surgical approaches is observed, which was accompanied by a reduced rate of severe hypothalamic lesions. Radical surgery is not an appropriate treatment strategy in patients with hypothalamic involvement. Despite previous recommendations to centralize treatment at specialized centers, a trend towards further decentralization was seen.

Zusammenfassung

Hintergrund: Die Prognose von Kindern und Jugendlichen mit Kraniopharyngeom wird durch Spätfolgen beeinträchtigt. Die radikale Resektion galt über Jahrzehnte als Therapie der Wahl. ­Allerdings drohen selbst bei Operation durch erfahrene neurochirurgische Teams hypothalami­sche Läsionen.

Fragestellung: Untersucht wurde, ob sich im Verlauf der Studien KRANIOPHARYNGEOM 2000/2007 Änderungen hinsichtlich der Behandlungs­strategie ergaben.

Patienten und Methoden: Verglichen wurden der Grad präoperativer hypothalamischer Beteiligung, operative Strategien, Resektionsgrad und Grad chirurgischer hypothalamischer Läsionen zwischen den Patientenkollektiven in KRANIO­PHARYNGEOM 2000 [n=120; 2001–2007) und KRANIOPHARYNGEOM 2007 (n=106; 2007–2012).

Ergebnisse: Es bestanden keine Unterschiede hinsichtlich der präoperativen hypothala­mischen Beteiligung zwischen beiden Studienkollektiven. Die Anzahl der radikalen Resektionen war höher (p=0.01) bei Patienten, die 2001–2007 rekrutiert wurden (38%), im Vergleich zum Rekrutierungs­zeitraum 2007–2012 (18%). Bei Risikopatienten mit präoperativer Beteiligung anteriorer/poste­riorer hypothalamischer Areale resultierte im Rekrutierungszeitraum 2007–2012 eine scho­nendere operative Vorgehendweise in einer höheren Rate von Patienten ohne operative Läsionen (35%) im Vergleich zum Rekrutierungs­zeitraum 2001–2007 (13%). Die ereignisfreie Überlebensrate war in beiden Studien gleich.

Schlussfolgerungen: Im Vergleich der Studien zeigt sich in den letzten 12 Jahren ein Trend hin zu weniger radikalen chirurgischen Behandlungsstrategien mit Reduktion schwerer hypo­tha­lamischer Läsionen. Bei Kraniopharyngeomen mit hypothalamischer Beteiligung erscheint die radikale Resektion nicht empfeh­­lenswert.

Key words

craniopharyngioma - hypothalamus - obesity - quality of life - pediatrics - neurooncology

Schlüsselwörter

Kraniopharyngeom - Hypothalamus - Adipositas - Lebensqualität - Pädiatrie - Neuroonkologie

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