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.
KeywordsJuvenile pilocytic astrocytoma (JPA) Pilomyxoid astrocytoma (PMA) Subependymal giant cell astrocytoma (SEGA) Neurofibromatosis (NF) Chemotherapy BRAF Malignant transformation
- 5.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.CrossRefPubMedCentralPubMedGoogle Scholar
- 6.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.CrossRefPubMedGoogle Scholar
- 11.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.CrossRefPubMedCentralPubMedGoogle Scholar
- 12.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.CrossRefPubMedCentralPubMedGoogle Scholar
- 14.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.CrossRefPubMedGoogle Scholar
- 21.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.CrossRefPubMedGoogle Scholar