Medizinische Universität Graz Austria/Österreich - Forschungsportal - Medical University of Graz
Gewählte Publikation:
SHR Neuro Krebs Kardio Lipid Stoffw Microb
Perwein, T; Benesch, M; Kandels, D; Pietsch, T; Schmidt, R; Quehenberger, F; Bison, B; Warmuth-Metz, M; Timmermann, B; Krauss, J; Thomale, UW; Kortmann, RD; Driever, PH; Gnekow, AK.
High frequency of disease progression in pediatric spinal cord low-grade glioma (LGG): management strategies and results from the German LGG study group.
Neuro Oncol. 2021; 23(7):1148-1162
Doi: 10.1093/neuonc/noaa296
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- Führende Autor*innen der Med Uni Graz
- Perwein Thomas
- Co-Autor*innen der Med Uni Graz
- Benesch Martin
- Quehenberger Franz
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- Abstract:
- BACKGROUND: Knowledge on management of pediatric spinal cord low-grade glioma (LGG) is scarce. METHODS: We analyzed clinical datasets of 128 pediatric patients with spinal LGG followed within the prospective multicenter trials HIT-LGG 1996 (n = 36), SIOP-LGG 2004 (n = 56), and the subsequent LGG-Interim registry (n = 36). RESULTS: Spinal LGG, predominantly pilocytic astrocytomas (76%), harbored KIAA1549-BRAF fusion in 14/35 patients (40%) and FGFR1-TACC1 fusion in 3/26 patients (12%), as well as BRAFV600E mutation in 2/66 patients (3%). 10-year overall survival (OS) and event-free survival (EFS) was 93% ± 2% and 38% ± 5%, respectively. Disseminated disease (n = 16) was associated with inferior OS and EFS, while age ≥11 years and total resection were favorable factors for EFS. We observed 117 patients following total (n = 24) or subtotal/partial resection (n = 74), biopsy (n = 16), or radiologic diagnosis only (n = 3). Eleven patients were treated first with chemotherapy (n = 9) or irradiation (n = 2). Up to 20.8 years after diagnosis/initial intervention, 73/128 patients experienced one (n = 43) or up to six (n = 30) radiological/clinical disease progressions. Tumor resections were repeated in 36 patients (range, 2-6) and 47 patients required nonsurgical treatment (chemotherapy, n = 20; radiotherapy, n = 10; multiple treatment lines, n = 17). Long-term disease control for a median of 6.5 (range, 0.02-20) years was achieved in 73/77 patients following one (n = 57) or repeated (n = 16) resections, and in 35/47 patients after nonsurgical treatment. CONCLUSIONS: The majority of patients experienced disease progression, even after years. Multiple interventions were required for more than a third, yet multimodal treatment enabled long-term disease control. Molecular testing may reveal therapeutic targets.
- Find related publications in this database (using NLM MeSH Indexing)
- Brain Neoplasms - genetics, therapy
- Child - administration & dosage
- Disease Progression - administration & dosage
- Glioma - genetics, therapy
- Humans - administration & dosage
- Prospective Studies - administration & dosage
- Spinal Cord - administration & dosage
- Find related publications in this database (Keywords)
- children
- low-grade gliomas
- spinal cord glioma
- surgery
- therapy