Klin Padiatr 2019; 231(03): 107-135
DOI: 10.1055/a-0889-8256
Diagnostic and Treatment Recommendation
© Georg Thieme Verlag KG Stuttgart · New York

SIOP-E-BTG and GPOH Guidelines for Diagnosis and Treatment of Children and Adolescents with Low Grade Glioma

SIOP-E BTG und GPOH Empfehlungen für die Diagnose und Behandlung von Kindern und Jugendlichen mit einem niedriggradigen Gliom
Astrid K. Gnekow
1   Swabian Children’s Cancer Center, University Hospital Augsburg, Augsburg
,
Daniela Kandels
1   Swabian Children’s Cancer Center, University Hospital Augsburg, Augsburg
,
Cornelis van Tilburg
2   Hopp Children’s Cancer Center Heidelberg (KiTZ), Heidelberg University Hospital, Heidelberg
3   Hopp Children's Cancer Center Heidelberg (KiTZ), German Cancer Research Center (DKFZ), Heidelberg, Germany
,
Amedeo A. Azizi
4   Department of Pediatrics and Adolescent Medicine, Medizinische Universitat Wien, Wien, Austria
,
Enrico Opocher
5   Department of Pediatric Oncology, Universita degli Studi di Padova, Padova, Italy
,
Tore Stokland
6   Department of Pediatrics, Universitetssykehuset Nord-Norge, Tromso, Norway
,
Pablo Hernaiz Driever
7   Pediatric Oncology and Hematology, Charité-Universitätsmedizin Berlin, Berlin
,
A. Y. N. Schouten-van Meeteren
8   Princess Máxima Children’s Cancer Center, Utrecht, Netherlands
,
Ulrich W. Thomale
9   Department of Pediatric Neuro-Surgery, Charité-Universitätsmedizin Berlin, Berlin
,
Martin U. Schuhmann
10   Division of Pediatric Neurosurgery, Department of Neurosurgery, University of Tuebingen, Tuebingen
,
Thomas Czech
11   Department of Neurosurgery, Medizinische Universitat Wien, Wien, Austria
,
John Robert Goodden
12   Department of Neurosurgery, Leeds General Infirmary & Leeds Children’s Hospital, Leeds, United Kingdom of Great Britain and Northern Ireland
,
Monika Warmuth-Metz
13   Institute of Diagnostic and Interventional Neuroradiology, University Hospital Wuerzburg, Wuerzburg
,
Brigitte Bison
13   Institute of Diagnostic and Interventional Neuroradiology, University Hospital Wuerzburg, Wuerzburg
,
Shivaram Avula
14   Department of Radiology, Alder Hey Children’s Hospital, Liverpool United Kingdom of Great Britain and Northern Ireland
,
Rolf-D. Kortmann
15   Department of Radio-Oncology, Universitätsklinikum Leipzig AöR, Leipzig
,
Beate Timmermann
16   Clinic for Particle Therapy at the Western German Proton Center, University Essen, Essen
,
Torsten Pietsch
17   Institute of Neuropathology, DGNN Brain Tumor Reference Center, University Bonn, Bonn
,
Olaf Witt
2   Hopp Children’s Cancer Center Heidelberg (KiTZ), Heidelberg University Hospital, Heidelberg
3   Hopp Children's Cancer Center Heidelberg (KiTZ), German Cancer Research Center (DKFZ), Heidelberg, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
20 May 2019 (online)

Abstract

Low grade gliomas (LGGs) constitute the largest, yet clinically and (molecular-) histologically heterogeneous group of pediatric brain tumors of WHO grades I and II occurring throughout all pediatric age groups and at all central nervous system (CNS) sites. The tumors are characterized by a slow growth rate and may show periods of growth arrest. Around 40% of all LGG patients can be cured by complete neurosurgical resection and are followed by close observation. In case of relapse, second resection often is possible. Following incomplete resection observation is recommended, as long as there is no radiologic tumor growth and the patient does not suffer from significant, tumor-related symptoms. This also applies to patients with a diagnosis of LGG on the basis of radiological criteria. By contrast, clinical worsening and / or radiologic progression are an indication to treatment with either chemo- or radiotherapy. Overall survival is around 90%, and many patients survive with residual tumor, i. e. they suffer from chronic disease. All patients need comprehensive neuro-oncological care, the principles and details of which are summarized in the current guidelines. These represent standard of care for diagnostic work-up (including neuroimaging and neuropathology), and for therapeutic decisions (including the indications to non-surgical treatment) as well as concepts for neurosurgical intervention, chemotherapy and radiotherapy as well as surveillance and rehabilitation. The current treatment algorithm was compiled by members of the LGG working group of the SIOP-E brain tumor group (SIOP-E-BTG) and is based upon the results of previous European LGG studies and international reports.

Zusammenfassung

Niedrigmaligne Gliome (LGG) WHO-Grad I und II stellen die größte, wenn auch klinisch und (molekular-)histologisch heterogene Gruppe pädiatrischer Hirntumore dar. Sie treten in sämtlichen pädiatrischen Altersgruppen und in allen Regionen des zentralen Nervensystems auf. Die Tumoren sind charakterisiert durch ihr langsames Wachstum und können auch Phasen des Wachstumsstillstands zeigen. Rund 40% aller LGG lassen sich komplett resezieren und es genügt, diese Patienten anschließend engmaschig zu beobachten. Im Falle eines Rezidivs ist häufig eine erneute Resektion möglich. Auch nach einer partiellen Resektion sollten Patienten lediglich beobachtet werden, solange der Tumor stabil ist und keine schweren tumorbedingten Symptome auftreten. Dies gilt auch für diejenigen Patienten, deren LGG lediglich auf der Grundlage radiologischer Kriterien diagnostiziert wurde. Eine Indikation für Chemo- oder Radiotherapie besteht hingegen bei klinischer und / oder radiologischer Progression. Das Gesamtüberleben liegt bei ca. 90%, viele Patienten weisen aber einen Resttumor auf. Um dem chronischen Verlauf dieser Tumorerkrankungen Rechnung zu tragen, benötigen die Patienten eine umfassende neuro-onkologische Betreuung, deren Prinzipien und Details in dem vorliegenden Artikel zusammengefasst werden. Dargestellt werden die aktuellen Standards für Diagnose (inklusive Bildgebung und Neuropathologie) und Therapie (inklusive Indikationsstellung für nicht-chirurgische Behandlungen) sowie die Konzepte der neurochirurgischen, chemo- und strahlentherapeutischen Interventionen und der Nachsorge und Rehabilitation. Der aktuelle Behandlungs-Algorithmus wurde von den Mitgliedern der LGG-Arbeitsgruppe der SIOP-E brain tumor group (SIOP-E-BTG) konsentiert und basiert auf den Ergebnissen der europäischen und internationalen LGG-Studien und -Publikationen.

 
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