Abstract
Low grade gliomas (LGG) represent a heterogeneous group of grade I and II tumors as per the WHO grading system with the majority of astrocytic origin. They are the most common tumor subtype within the pediatric population. Location is influenced by age and underlying neurocutaneous syndromes (e.g., neurofibromatosis, tuberous sclerosis); the most common location is within the posterior fossa, followed by the optic pathway. Given the slow growth rate, LGG should be treated as a chronic disease with less aggressive treatment. Sometimes, potentially driven by young age, low grade gliomas can show a fast growth rate without a change in histology. Also spontaneous regression has been observed, particularly in those with neurofibromatosis type I. If surgical removal is not possible, mainly in midline location tumors, chemotherapy is the treatment of choice. Treatment indication can be challenging sometimes and is influenced by clinical symptoms and imaging findings. Often a wait-and-observe strategy is also a treatment option. Long-term survival is high (>90 %), but the progression-free survival with residual disease is around 50 %, and the long-term sequelae rate is high. Malignant transformation to a high grade glioma is seen very rarely compared to the adult population.
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References
Sievert AJ, Fisher MJ. Pediatric low-grade gliomas. J Child Neurol. 2009;24(11):1397–408.
Panigrahy A, Blűml S. Neuroimaging of pediatric brain tumors: from basic to advanced magnetic resonance imaging (MRI). J Child Neurol. 2009;24(11):1343–65.
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:97–109.
Tihan T, Fisher PG, Kepner JL, Godfraind C, McComb RD, Goldthwaite PT, Burger PC. Pediatric astrocytomas with monomorphous pilomyxoid features and a less favourable outcome. J Neuropathol Exp Neurol. 1999;58:1061–8.
Pfister S, Janzarik WG, Remke M, Ernst A, Werft W, Becker N, Toedt G, Wittmann A, Kratz C, Olbrich H, Ahmadi R, Thieme B, Joos S, Radlwimmer B, Kulozik A, Pietsch T, Herold-Mende C, Gnekow A, Reifenberger G, Korshunov A, Scheurlen W, Omran H, Lichter P. BRAF gene duplication constitutes a mechanism of MAPK pathway activation in low-grade astrocytomas. J Clin Invest. 2008;118(5):1739–49.
Hawkins C, Walker E, Mohamed N, Zhang C, Jacob K, Shirinian M, Alon N, Kahn D, Fried I, Scheinemann K, Tsangaris E, Dirks P, Tressler R, Bouffet E, Jabado N, Tabori U. BRAF-KIAA1549 fusion predicts better clinical outcome in pediatric low-grade astrocytoma. Clin Cancer Res. 2011;17(14):4790–8.
Tabori U, Vukovic B, Zielenska M, Hawkins C, Braude I, Rutka J, Bouffet E, Shire J, Malkin D. The role of telomere maintenance in the spontaneous growth arrest of pediatric low-grade gliomas. Neoplasia. 2006;8(2):136–42.
Wisoff JH, Sanford RA, Heier LA, Sposto R, Burger PC, Yates AJ, Homes EJ, Kun LE. Primary neurosurgery for Pediatric low-grade gliomas: a prospective multi-institutional study from the Children’s Oncology Group. Neurosurgery. 2011;68(6):1548–54.
Merchant TE, Conklin HM, Wu S, Lustig RH, Xiong X. Late effects of conformal radiation therapy for pediatric patients with low-grade glioma: prospective evaluation of cognitive, endocrine and hearing deficits. J Clin Oncol. 2009;27(22):3691–7.
Merchant TE, Kun LE, Wu S, Xiong X, Sanford RA, Boop FA. Phase II trial of conformal radiation therapy for pediatric low-grade glioma. J Clin Oncol. 2009;27(22):3598–604.
Ater JL, Zhou T, Holmes E, Mazewski CM, Booth TN, Freyer DR, Lazarus KH, Packer RJ, Prados M, Sposto R, Vezina G, Wisoff JH, Pollack IF. Randomized study of two chemotherapy regimens for treatment of low-grade glioma in young children: a report from the Children’s Oncology Group. J Clin Oncol. 2012;30(21):2641–7.
Gnekow AK, Falkenstein F, von Hornstein S, Zwiener I, Berkefeld S, Bison S, Warmuth-Metz M, Hernaiz Driver P, Soerensen N, Kortmann RD, Pietsch T, Faldum A. 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. 2012;14(10):1265–84.
Lafay-Cousin L, Holm S, Qaddoumi I, Nicolin G, Bartels U, Tabori U, Huang A, Bouffet E. Weekly vinblastine in pediatric low-grade glioma patients with carboplatin allergic reaction. Cancer. 2005;103(12):2636–42.
Bouffet E, Jakacki R, Goldman S, Hargrave D, Hawkins C, Shroff M, Hukin J, Bartels U, Foreman N, Kellie S, Hilden J, Etzel M, Wilson B, Stephens D, Tabori U, Baruchel S. Phase II study of weekly vinblastine in recurrent or refractory pediatric low-grade glioma. J Clin Oncol. 2012;30(12):1358–63.
Scheinemann K, Bartels U, Tsangaris E, Hawkins C, Huang A, Dirks P, Fried I, Bouffet E, Tabori U. Feasibility and efficacy of repeated chemotherapy for progressive pediatric low-grade gliomas. Pediatr Blood Cancer. 2011;57(1):84–8.
Hwang EI, Jakacki RI, Fisher MJ, Kilburn LB, Horn M, Vezina G, Rood BR, Packer RJ. Long-term efficacy and toxicity of bevacizumab-based therapy in children with recurrent low-grade gliomas. Pediatr Blood Cancer. 2013;60:776–82.
Lam C, Bouffet E, Tabori U, Mabbott D, Taylor M, Bartels U. Rapamycin (sirolimus) in tuberous sclerosis associated pediatric central nervous system tumors. Pediatr Blood Cancer. 2010;54(3):476–9.
Scheinemann K, Bartels U, Huang A, Hawkins C, Kulkarni AV, Bouffet E, Tabori U. Survival and functional outcome of childhood spinal cord low-grade gliomas. J Neurosurg Pediatr. 2009;4(3):254–61.
Hernaiz Driever P, von Hornstein S, Pietsch T, Kortmann R, Warmuth-Metz M, Emser A, Gnekow AK. Natural history and management of low-grade glioma in NF-1 children. J Neurooncol. 2010;100:199–207.
Broniscer A, Baker SJ, West AN, Fraser MM, Proko E, Kocak M, Dalton J, Zambetti GP, Ellison DW, Kun LE, Gajjar A, Gilbertson RJ, Fuller CE. Clinical and molecular characteristics of malignant transformation of low-grade glioma in children. J Clin Oncol. 2007;28(6):682–9.
Benesch M, Lackner H, Sovinz P, Suppan E, Schwinger W, Eder HG, Dombusch HJ, Moser A, Triebl-Roth K, Urban C. Late sequelae after treatment of childhood low-grade gliomas: a retrospective analysis of 69 long-term survivors treated between 1983 and 2003. J Neurooncol. 2006;78:199–205.
Ris MD, Beebe DW. Neurodevelopmental outcomes of children with low-grade gliomas. Dev Disabil Res Rev. 2008;14:196–202.
Musial-Bright L, Panteli L, Driver Hernaiz P. Pediatric low-grade glioma survivors experience high quality of life. Childs Nerv Syst. 2011;27:1895–902.
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Scheinemann, K., Hukin, J. (2015). Low Grade Glioma. In: Scheinemann, K., Bouffet, E. (eds) Pediatric Neuro-oncology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1541-5_9
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DOI: https://doi.org/10.1007/978-1-4939-1541-5_9
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