Favorable prognosis in pediatric brainstem low-grade glioma: Report from the German SIOP-LGG 2004 cohort |
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| Authors: | Johannes Holzapfel Daniela Kandels René Schmidt Torsten Pietsch Monika Warmuth-Metz Brigitte Bison Jüergen Krauss Rolf-Dieter Kortmann Beate Timmermann Ulrich-Wilhelm Thomale Michael H. Albert Pablo Hernáiz Driever Olaf Witt Astrid K. Gnekow |
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| Affiliation: | 1. Swabian Children's Cancer Center, University Hospital Augsburg, Augsburg, Germany;2. Institute of Biostatistics and Clinical Research, University of Muenster, Münster, Germany;3. Institute of Neuropathology, DGNN Brain Tumor Reference Center, University Bonn, Bonn, Germany;4. Institute of Diagnostic and Interventional Neuroradiology, University Hospital Wuerzburg, Wuerzburg, Germany;5. Section of Pediatric Neurosurgery, University Hospital Wuerzburg, Wuerzburg, Germany;6. Department of Radio-Oncology, University Leipzig, Leipzig, Germany;7. Department of Particle Therapy, University Hospital Essen, West German Proton Therapy Centre Essen (WPE), West German Cancer Center (WTZ), German Cancer Consortium (DKTK), Essen, Germany;8. Pediatric Neurosurgery, Charité Universitaetsmedizin Berlin, Berlin, Germany;9. Dr. von Hauner Children's Hospital, Ludwig-Maximilians Universitaet, Munich, Germany;10. Department of Pediatric Oncology/Hematology, Charité Universitaetsmedizin Berlin, Corporate member of Freie Universitaet Berlin, Humboldt-Universitaet zu Berlin, and Berlin Institute of Health, Berlin, Germany;11. Hopp Children's Cancer Center Heidelberg (KiTZ), German Cancer Research Center (DKFZ) and Heidelberg University Hospital, Heidelberg, Germany |
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| Abstract: | Reports on pediatric low-grade glioma (LGG) of the caudal brainstem are retrospective with heterogeneous cohorts, variable treatments and inconsistent outcome data. We analyzed their natural history and asked whether brainstem location proved unfavorable for survival within the framework of the comprehensive SIOP-LGG 2004 management strategy. Within the prospectively registered, population-based German SIOP-LGG 2004 cohort 116 patients (age 0.2–16.5 years, 10% Neurofibromatosis NF1) were diagnosed with LGG of the pons (27%) and medulla oblongata (73%). After biopsy (23%), variable resection (63%) or radiologic diagnosis only (14%), 59 patients received no adjuvant treatment. Radiologic progression or severe neurologic symptoms prompted chemo- (n = 39) or radiotherapy (n = 18). After further progression (28/57), salvage treatments included multiple treatment lines for 12/28 patients. Five-years event-free survival dropped to 0.40, while 5-years overall survival was 0.95 (median observation time 6.8 years). Higher extent of resection yielded lower progression rate (p = 0.001), but at a cost of 21/100 patients suffering from new postsurgical complications including respiratory insufficiency. Central review confirmed pilocytic astrocytoma (56%), diffuse astrocytoma (8%) or glioneuronal histology (16%) (others 4%, no histology 17%). Malignant evolution was documented in five patients associated with Histone3 mutation in 2/5. Our treatment algorithm conveyed high overall survival for pediatric brainstem LGG. Extensive neurosurgical resection did increase additional postoperative neurologic deficits but not overall survival in this often-chronic disease. More than half of all patients can be safely followed by observation, while multimodal adjuvant treatment can control progressive tumors. Molecular assessment should confirm low-grade diagnosis and may detect patterns prognostic for malignant evolution. |
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| Keywords: | brainstem low grade glioma child surgery chemotherapy radiotherapy |
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