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Neuropediatrics 2018; 49(05): 314-323
DOI: 10.1055/s-0038-1660503
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Management of Primary Tectal Plate Low-Grade Glioma in Pediatric Patients: Results of the Multicenter Treatment Study SIOP-LGG 2004

Ariane Kaufmann   *
1   Department of Oncology, University Children's Hospital, Zurich, Switzerland
,
Nicolas U. Gerber   *
1   Department of Oncology, University Children's Hospital, Zurich, Switzerland
,
Daniela Kandels
2   Hospital for Children and Adolescents, Klinikum Augsburg, Augsburg, Bayern, Germany
,
Amedeo A. Azizi
3   Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Rene Schmidt
4   Institute of Biostatistics and Clinical Research, University of Muenster, Muenster, Germany
,
Monika Warmuth-Metz
5   Department of Neuroradiology, University of Wuerzburg, Wuerzburg, Germany
,
Torsten Pietsch
6   Institute of Neuropathology, University of Bonn, Bonn, Germany
,
Rolf-Dieter Kortmann
7   Department of Radiation Oncology, University of Leipzig, Leipzig, Germany
,
Astrid K. Gnekow
2   Hospital for Children and Adolescents, Klinikum Augsburg, Augsburg, Bayern, Germany
,
Michael A. Grotzer
1   Department of Oncology, University Children's Hospital, Zurich, Switzerland
› Author Affiliations Funding The German SIOP-LGG 2004 trial center in Augsburg, Germany, the Reference Center for Neuropathology, Institute of Neuropathology, University of Bonn, Germany, and the Reference Center for Biostatistics, Institute of Biostatistics and Clinical Research, University of Muenster, Germany, were supported by grants from the German Children's Cancer Foundation (DKKS).
Further Information

Publication History

05 February 2018

26 April 2018

Publication Date:
11 June 2018 (online)

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Abstract

Background Tectal plate low-grade gliomas (LGGs) most often present with increased intracranial pressure and sometimes as incidental findings from brain imaging. Prognostic factors predicting outcome are largely unknown.

Methods From 2004 until 2012, 71 patients with tectal plate LGG from Germany and Switzerland were followed within the SIOP-LGG 2004 study. Median age at diagnosis was 9.7 (range: 0.1–17.5) years, and median follow-up time of surviving patients was 6.3 (interquartile range: 4.9–8.3) years.

Results A total of 41 out of 71 patients received no tumor treatment (12 with and 29 without biopsy). The 10-year event-free survival (EFS) rate (± standard error ) for patients with an initial tumor volume of ≤3 cm3 was 56% (±7%), as opposed to 12% (±8%) for those with tumors >3 cm3 (p < 0.001). The 10-year EFS for patients without contrast enhancement on initial magnetic resonance imaging (MRI) was 52% (±9%), and for those with enhancement, it was 23% (±9%) (p = 0.003). The 10-year overall survival rate was 96% (±3%) (death due to disease, 1; ventriculoperitoneal shunt infection, 1). Sixty-three (89%) patients had at least one cerebrospinal fluid diversion procedure.

Conclusions More than half of patients were managed without tumor treatment. Favorable prognostic factors for EFS were small initial tumor volume (≤3cm3) and the absence of initial contrast enhancement on MRI. Overall survival was excellent.

Keywords

tectal plate glioma - tectal glioma - low-grade glioma - astrocytoma - brain tumor - children

* Ariane Kaufmann and Nicolas U. Gerber contributed equally to this work.


Supplementary Material

 

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