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Low Grade Glioma

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.

Keywords

Juvenile pilocytic astrocytoma (JPA) Pilomyxoid astrocytoma (PMA) Subependymal giant cell astrocytoma (SEGA) Neurofibromatosis (NF) Chemotherapy BRAF Malignant transformation 

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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Department of Pediatrics, Division of Hematology/OncologyMcMaster Children’s HospitalMcMaster University, HamiltonCanada
  2. 2.Division of Neurology and OncologyBritish Columbia Children’s HospitalVancouverCanada

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