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Klin Padiatr 2023; 235(03): 167-177
DOI: 10.1055/a-2070-7572
Originalarbeit

Kinder und Jugendliche mit intrakraniellem Ependymom – Empfehlungen der HIT-MED-Studiengruppe der GPOH zur Erstlinientherapie

Pediatric Intracranial Ependymoma – Recommendations for First-Line Treatment from the German HIT-MED study group
Denise Obrecht 
1   Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Martin Mynarek
1   Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Martina Stickan-Verfürth
2   Department of Particle Therapy, West German Proton Therapy Centre Essen (WPE), University Hospital Essen, Essen, Germany
,
Brigitte Bison
3   Diagnostic and Interventional Neuroradiology, Faculty of Medicine, University of Augsburg, Augsburg, Germany
,
Ulrich Schüller
4   Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
5   Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
6   Research Institute Children’s Cancer Center Hamburg, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Kristian Pajtler
7   Hopp Children’s Cancer Center Heidelberg (KiTZ), University Hospital Heidelberg, Heidelberg, Germany
8   Division of Pediatric Neurooncology, German Cancer Research Center (DKFZ) and German Cancer Consortium (DKTK), University Hospital Heidelberg, Heidelberg, Germany
9   Department of Pediatric Oncology, Hematology and Immunology, University Hospital Heidelberg, Heidelberg, Germany
,
Christian Hagel
5   Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
,
Ulrich-Wilhelm Thomale
10   Department of Neurosurgery, Section of pediatric Neurosurgery, Charité Universitätsmedizin Berlin, Berlin, Germany
,
Gudrun Fleischhack 
11   Pediatrics III, University Hospital Essen, Essen, Germany
,
Beate Timmermann
2   Department of Particle Therapy, West German Proton Therapy Centre Essen (WPE), University Hospital Essen, Essen, Germany
,
Stefan Rutkowski
1   Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
› Author Affiliations Funding Information Deutsche Kinderkrebsstiftung — http://dx.doi.org/10.130 39/501100007311; Fördergemeinschaft Kinderkrebs-Zentrum Hamburg — http://dx.doi.org/10.13039/501100006146;
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Zusammenfassung

In den letzten Jahren wurden biologische Subgruppen des Ependymoms (EPN) identifiziert und in die aktuelle WHO-Klassifikation eingeführt, allerdings haben diese derzeit noch keinen Einfluss auf die Therapiestratifizierung. Jedoch finden sich immer mehr Hinweise, dass das klinische Verhalten abhängig von der Tumorbiologie ist: PF-EPN-A mit einem Zugewinn des Chromosom 1q beispielsweise sind mit einem hohen Risiko für Rezidive assoziiert, während das Überleben bei ST-EPN-YAP1 bei>90% liegt. Die insgesamt noch nicht zufriedenstellende Prognose zeigt, dass die Therapiestrategien weiter verbessert werden müssen. Das Ausmaß der Tumorresektion ist der wichtigste klinische Prognosefaktor, weshalb die Evaluation einer erneuten OP bei einem postoperativen Resttumor höchste Priorität hat. Darüber hinaus wird die Wirksamkeit der Lokalbestrahlung als bewiesen angesehen und derzeit im Alter>1 Jahr empfohlen. Der Stellenwert der Chemotherapie ist weiterhin unklar. Ein Ziel der europäischen klinischen Studie SIOP Ependymoma II ist, die Wirksamkeit verschiedener Chemotherapien zu evaluieren, so dass deutsche Patienten im Rahmen dieser Studie behandelt werden sollten, um den Kenntnisstand voranzutreiben. Neue prognostische biologische Parameter werden im Rahmen des Begleitprojekts BIOMECA validiert. Die Erkenntnisse dieser Begleitstudie sollen auch genutzt werden, um zielgerichtete Therapien sog. Targeted Therapies zu entwickeln. Für Patienten, die nicht im Rahmen der interventionellen Strata der Studie behandelt werden können, stellt die HIT-MED Guidance (aktuelle Version 5.2) eine Handlungsempfehlung dar. Der vorliegende Artikel gibt einen Überblick über Empfehlungen zu Diagnostik und Therapie für Kinder- und Jugendliche mit intrakraniellem Ependymom, sowie zur Behandlung im Rahmen der SIOP Ependymoma II Studie.

Abstract

Biological subtypes of ependymoma (EPN) have been introduced by the recent WHO classification and appear to have great impact on the clinical course, but have not yet found their way into clinical risk stratification. Further, the overall unfavorable prognosis underlines the fact that current therapeutic strategies need further evaluation for improvement. To date, there is no international consensus regarding first-line treatment for children with intracranial EPN. Extent of resection is known to be the most important clinical risk factor, leading to the consensus that consequent evaluation for re-surgery of postoperative residual tumor needs to have highest priority. Furthermore, efficacy of local irradiation is unquestioned and recommended for patients aged>1 year. In contrast, efficacy of chemotherapy is still under discussion. The European trial SIOP Ependymoma II aims at evaluating efficacy of different chemotherapy elements, leading to the recommendation to include German patients. The BIOMECA study, as biological accompanying study, aims at identifying new prognostic parameters. These results might help to develop targeted therapies for unfavorable biological subtypes. For patient who are not qualified for inclusion into the interventional strata, the HIT-MED Guidance 5.2 provides specific recommendations. This article is meant as an overview of national guidelines regarding diagnostics and treatment as well as of treatment according to the SIOP Ependymoma II trial protocol.

Schlüsselwörter

Ependymom - Therapieempfehlung - Radiotherapie - Chemotherapie - Kinder

Key words

ependymoma - guideline - radiotherapy - chemotherapy - children


Publication History

Article published online:
12 May 2023

© 2023. Thieme. All rights reserved.

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