Abstract
Patients with disseminated pediatric low-grade glioma (LGG) initially treated with chemotherapy frequently experience disease progression, with 5-year event-free survival (EFS) of <20% and 10-year overall survival (OS) of approximately 70%. This study aimed to describe outcomes of metastatic pediatric LGG treated with craniospinal irradiation (CSI). A retrospective study was performed of all patients with metastatic pediatric LGG treated with CSI at a single institution. EFS was defined as survival without disease progression or secondary high-grade glioma. Dates were counted from the first day of irradiation. We identified 12 eligible patients; all had histologically confirmed LGG. Metastatic disease was present at initial presentation in 9 patients. The median age at CSI was 9.3 years. The 5-year EFS and OS were 71% (95% CI 33.7–89.5) and 70% (95% CI 32.9–89.2), respectively. No deaths were observed among the patients who underwent subtotal resection (STR) before radiotherapy, whereas 3 patients who had undergone biopsy died (OS log-rank P = 0.01). EFS may be longer among patients who underwent STR before RT (EFS log-rank P = 0.03), with a hazard ratio for biopsy of 8.4 (vs. STR; 95% CI 0.8–84.0, P = 0.07). No patient experienced acute toxicity of grade 3 or higher. Patients with metastatic pediatric LGG treated with CSI experienced longer EFS than historical cohorts treated with chemotherapy alone, with similar OS. CSI may be considered in the management of metastatic pediatric LGG, particularly in older children experiencing progression after chemotherapy.
Similar content being viewed by others
References
Hukin J, Siffert J, Cohen H, Velasquez L, Zagzag D, Allen J (2003) Leptomeningeal dissemination at diagnosis of pediatric low-grade neuroepithelial tumors. Neuro Oncol 5:188–196. doi:10.1215/S1152-8517-02-00029-7
Hukin J, Siffert J, Velasquez L, Zagzag D, Allen J (2002) Leptomeningeal dissemination in children with progressive low-grade neuroepithelial tumors. Neuro Oncol 4:253–260
Perilongo G, Garre ML, Giangaspero F (2003) Low-grade gliomas and leptomeningeal dissemination: a poorly understood phenomenon. Childs Nerv Syst 19:197–203. doi:10.1007/s00381-003-0733-1
Chamdine O, Broniscer A, Wu S, Gajjar A, Qaddoumi I (2016) Metastatic low-grade gliomas in children: 20 years’ experience at St. Jude Children’s Research Hospital. Pediatr Blood Cancer 63:62–70. doi:10.1002/pbc.25731
von Hornstein S, Kortmann RD, Pietsch T, Emser A, Warmuth-Metz M, Soerensen N, Straeter R, Graf N, Thieme B, Gnekow AK (2011) Impact of chemotherapy on disseminated low-grade glioma in children and adolescents: report from the HIT-LGG 1996 trial. Pediatr Blood Cancer 56:1046–1054. doi:10.1002/pbc.23006
Gajjar A, Bhargava R, Jenkins JJ, Heideman R, Sanford RA, Langston JW, Walter AW, Kuttesch JF, Muhlbauer M, Kun LE (1995) Low-grade astrocytoma with neuraxis dissemination at diagnosis. J Neurosurg 83:67–71. doi:10.3171/jns.1995.83.1.0067
Gnekow AK, Falkenstein F, von Hornstein S, Zwiener I, Berkefeld S, Bison B, Warmuth-Metz M, Driever PH, Soerensen N, Kortmann RD, Pietsch T, Faldum A (2012) Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology. Neuro Oncol 14:1265–1284. doi:10.1093/neuonc/nos202
Qaddoumi I, Sultan I, Gajjar A (2009) Outcome and prognostic features in pediatric gliomas: a review of 6212 cases from the Surveillance, Epidemiology, and End Results database. Cancer 115:5761–5770. doi:10.1002/cncr.24663
Packer RJ, Pfister S, Bouffet E, Avery R, Bandopadhayay P, Bornhorst M, Bowers DC, Ellison D, Fangusaro J, Foreman N, Fouladi M, Gajjar A, Haas-Kogan D, Hawkins C, Ho CY, Hwang E, Jabado N, Kilburn LB, Lassaletta A, Ligon KL, Massimino M, Meeteren SV, Mueller S, Nicolaides T, Perilongo G, Tabori U, Vezina G, Warren K, Witt O, Zhu Y, Jones DT, Kieran M (2016) Pediatric low-grade gliomas: implications of the biologic era. Neuro Oncol. doi:10.1093/neuonc/now209
Acknowledgements
This work was supported by the American Cancer Society (Grant Number: SPAMM-15-210-01-COUN) and the American Lebanese Syrian Associated Charities (ALSAC). The authors would like to thank Roletta Ammons, MBA and Tina Davis, MBA for assistance with data collection, and Keith A. Laycock, PhD, ELS, for scientific editing of the manuscript.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors declare that they have no conflict of interest.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Tsang, D.S., Murphy, E.S., Ezell, S.E. et al. Craniospinal irradiation for treatment of metastatic pediatric low-grade glioma. J Neurooncol 134, 317–324 (2017). https://doi.org/10.1007/s11060-017-2525-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11060-017-2525-6