High frequency of disease progression in pediatric spinal cord low-grade glioma (LGG): management strategies and results from the German LGG study group

被引:13
作者
Perwein, Thomas [1 ]
Benesch, Martin [1 ]
Kandels, Daniela [2 ]
Pietsch, Torsten [3 ]
Schmidt, Rene [4 ]
Quehenberger, Franz [5 ]
Bison, Brigitte [6 ]
Warmuth-Metz, Monika [6 ]
Timmermann, Beate [7 ]
Krauss, Jurgen [8 ]
Thomale, Ulrich-Wilhelm [9 ]
Kortmann, Rolf-Dieter [10 ]
Driever, Pablo Hernaiz [11 ,12 ,13 ,14 ]
Gnekow, Astrid Katharina [2 ]
机构
[1] Med Univ Graz, Dept Pediat & Adolescent Med, Div Pediat Hematol & Oncol, Auenbruggerpl 38, A-8036 Graz, Austria
[2] Univ Hosp Augsburg, Swabian Childrens Canc Ctr, Augsburg, Germany
[3] Univ Bonn, Brain Tumor Reference Ctr, Inst Neuropathol, German Soc Neuropathol & Neuroanat DGNN, Bonn, Germany
[4] Univ Munster, Inst Biostat & Clin Res, Munster, Germany
[5] Med Univ Graz, Inst Med Informat Stat & Documentat, Graz, Austria
[6] Univ Wurzburg, Inst Diagnost & Intervent Neuroradiol, Wurzburg, Germany
[7] Essen Univ Hosp, West German Proton Therapy Ctr Essen, Clin Particle Therapy, Essen, Germany
[8] Univ Hosp Wurzburg, Sect Pediat Neurosurg, Wurzburg, Germany
[9] Charite Univ Med Berlin, Pediat Neurosurg, Berlin, Germany
[10] Univ Leipzig, Dept Radiooncol, Leipzig, Germany
[11] Charite Univ Med Berlin, Dept Pediat Oncol Hematol, Berlin, Germany
[12] Free Univ Berlin, Berlin, Germany
[13] Humboldt Univ, Berlin, Germany
[14] Berlin Inst Hlth, Berlin, Germany
关键词
children; low-grade gliomas; spinal cord glioma; surgery; therapy; PROGNOSTIC-FACTORS; TUMORS; CHILDREN; OUTCOMES; ASTROCYTOMAS; MULTICENTER; ONCOLOGY; ADOLESCENTS; DIAGNOSIS; SOCIETY;
D O I
10.1093/neuonc/noaa296
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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.
引用
收藏
页码:1148 / 1162
页数:15
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