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Hohe Rückfallraten nach Kraniopharyngeom im Kindes- und Jugendalter

Neue Therapiestrategien in KRANIOPHARYNGEOM 2007

High recurrence rates after craniopharyngioma in children and adolescents

Innovative therapeutic strategies in KRANIOPHARYNGEOM 2007

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Monatsschrift Kinderheilkunde Zeitschrift für Kinder- und Jugendmedizin Aims and scope Submit manuscript

Zusammenfassung

In der Querschnittsstudie HIT-ENDO wurden 258 Kinder und Jugendliche mit Kraniopharyngeom hinsichtlich Therapie und Langzeitprognose analysiert. Die prospektive Beobachtungsstudie KRANIOPHARYNGEOM 2000 untersuchte die Rezidivraten nach kompletter und die Progressionsraten nach inkompletter Resektion des Kraniopharyngeoms. Zwischen 10/2001 und 06/2006 wurden 98 Patienten rekrutiert und prospektiv untersucht. 24 wurden bestrahlt, in einem medianen Alter von 11,5 Jahren (4–18 Jahre), im Mittel 10 Monate nach der Diagnose. Eine Zwischenauswertung zur ereignisfreien Überlebenszeit (EFS nach 3 Jahren) ergab hohe Raten an frühen Ereignissen im Sinne von Tumorprogressionen nach subtotaler Resektion (3-Jahres-EFS=0,22±0,087) bzw. Rezidiven nach kompletter Resektion (3-Jahres-EFS=0,60±0,098). Wir schlussfolgern, dass Rezidive nach kompletter Resektion bzw. Tumorprogression nach inkompletter Resektion häufige Ereignisse während der ersten 3 Jahre darstellen. Regelmäßige Kontrollen der Bildgebung und des klinischen Status insbesondere in den ersten Jahren nach der Diagnose sind zu empfehlen. In KRANIOPHARYNGEOM 2007 wird für Patienten eines Alters von 5 Jahren und älter nach inkompletter Resektion die Frage des Zeitpunkts der Strahlentherapie (XRT) im Rahmen einer stratifizierten Randomisation geprüft (XRT direkt postoperativ vs. XRT bei Progression). Endpunkte der Analyse sind die gesundheitsbezogene Lebensqualität (PEDQOL-Domaine: physische Funktion), EFS und Gesamtüberleben.

Abstract

In the cross-sectional study HIT-ENDO, data on the therapy and prognosis of 258 patients with childhood craniopharyngioma (CP) were analyzed. Aims of the prospective study KRANIOPHARYNGEOM 2000 were to collect data on the incidence and time course of relapses after complete surgery and of tumor progressions after incomplete resection. Between October 2001 and June 2006, 98 patients with CP were recruited. Complete resection was achieved in 43% and subtotal resection in 45%. Irradiation (XRT) was performed in 24 of 98 CP patients at a median age of 11.5 years (range 4–18 years) and after a mean interval of 10 months after first diagnosis. Analysis of event-free survival rates (EFS) showed a high rate of early events in terms of tumor progression after incomplete resection (3-year EFS =0.22±0.087) and relapses after complete resection (3-year EFS =0.60±0.098) during the first 3 years of follow-up. We conclude that tumor progression and relapse are frequent and early events. Monitoring of cerebral imaging and clinical status is recommended in the follow-up of patients with childhood CP. To analyze the appropriate time point for XRT after incomplete resection, health-related quality of life, EFS, and overall survival in patients (at age of 5 years or older) will be analyzed in KRANIOPHARYNGEOM 2007 after stratified randomization of the time point for irradiation after incomplete resection (immediate XRT versus XRT at progression of residual tumor).

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Interessenkonflikt

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Authors and Affiliations

  1. Klinik für Allgemeine Kinderheilkunde, Hämatologie/Onkologie, Zentrum für Kinder- und Jugendmedizin, Klinikum Oldenburg gGmbH, Oldenburg, Dr.-Eden-Straße 10, 26133, Oldenburg, Deutschland

    H.L. Müller, U. Gehardt & R. Kolb

  2. Poliklinik für Strahlentherapie und Radioonkologie, Universitätsklinikum Leipzig, Leipzig, Deutschland

    R.D. Kortmann

  3. Klinik für Strahlentherapie, Universitätsklinikum Regensburg, Regensburg, Deutschland

    F. Pohl

  4. Klinik für Strahlentherapie, Universitätsklinik Würzburg, Würzburg, Deutschland

    M. Flentje

  5. Abteilung für Neuroradiologie, Universitätsklinik Würzburg, Würzburg, Deutschland

    M. Warmuth-Metz

  6. Institut für Medizinische Biometrie, Epidemiologie und Informatik, IMBEI, Universität Mainz, Mainz, Deutschland

    A. Emser

  7. Universitätskinderklinik Düsseldorf, Düsseldorf, Deutschland

    A. Wiener & G. Calaminus

  8. Abteilung für Pädiatrische Neurochirurgie, Neurochirurgische Universitätsklinik Würzburg, Würzburg, Deutschland

    N. Sörensen

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  1. H.L. Müller
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  2. U. Gehardt
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  3. R.D. Kortmann
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  4. F. Pohl
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  5. M. Flentje
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  6. M. Warmuth-Metz
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  7. A. Emser
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  8. R. Kolb
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  9. A. Wiener
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  10. G. Calaminus
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  11. N. Sörensen
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Correspondence to H.L. Müller.

Additional information

Im Namen der Studienkommissionen von KRANIOPHARYNGEOM 2000 und KRANIOPHARYNGEOM 2007.

Gefördert von der Deutschen Kinderkrebsstiftung, Bonn (http://www.kinderkrebsstiftung.de)

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Müller, H., Gehardt, U., Kortmann, R. et al. Hohe Rückfallraten nach Kraniopharyngeom im Kindes- und Jugendalter. Monatsschr Kinderheilkd 156, 155–161 (2008). https://doi.org/10.1007/s00112-007-1627-1

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