Summary and Further Reading

Chapter 11 - Classification and Treatment of Paediatric Brain Tumours


  • Brain tumours are the most frequent solid tumours in children, with embryonal tumours and LGGs representing the largest fraction of these cancers
  • Symptoms are related to age and tumour location
  • Approximately 10% of brain tumours occur in children with genetic syndromes, such as NF1 and NF2, Li-Fraumeni, Cowden, Gorlin and Turcot syndromes, tuberous sclerosis complex and Von Hippel-Lindau syndrome
  • Biopsy is very important to define diagnosis and individualised treatment based on molecular biology
  • In MB, four molecular subgroups have recently been identified, which are important for prognosis, risk stratification and treatment
  • LGGs are slow-growing tumours, with a relatively benign histology, and gross total excision is the main treatment. The most frequent genomic alterations in paediatric LGGs are KIAA-BRAF fusion genes or BRAF V600 Mutations
  • OPGs account for 3%–5% of paediatric intracranial tumours, and children with NF1 have an approximately 20% risk of developing an optic glioma
  • HGGs are clinically aggressive and regionally invasive. Current treatment consists of maximal resection and RT. The role of ChT is evolving
  • The prognosis in children with brainstem glioma remains poor. RT is the standard care, leading to temporary clinical improvement. Chemotherapeutic strategies, including multiagent neoadjuvant ChT, concurrent ChT with RT, and adjuvant ChT, have not provided any survival advantage
  • Two-thirds of ependymomas occur in the posterior fossa. The primary treatment remains surgical resection followed by RT, with gross total resection frequently reported as the most important Prognostic factor

Further Reading

Collins VP, Jones DT, Giannini C. Pilocytic astrocytoma: pathology, molecular mechanisms and markers. Acta Neuropathol 2015; 129:775–788.

Gajjar A, Bowers DC, Karajannis MA, et al. Pediatric brain tumors: innovative genomic information is transforming the diagnostic and clinical landscape. J Clin Oncol 2015; 33:2986–2998.

Gerber NU, Mynarek M, von Hoff K, et al. Recent developments and current concepts in medulloblastoma. Cancer Treat Rev 2014; 40:356–365.

Lin FY, Chintagumpala M. Advances in management of pediatric ependymomas. Curr Oncol Rep 2015; 17:47.

Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 2016; 131:803–820.

Northcott PA, Korshunov A, Pfister SM, Taylor MD. The clinical implications of medulloblastoma subgroups. Nat Rev Neurol 2012; 8:340–351.

Pajtler KW, Witt H, Sill M, et al. Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell 2015; 27:728–743.

Pietsch T, Schmidt R, Remke M, et al. Prognostic significance of clinical, histopathological, and molecular characteristics of medulloblastomas in the prospective HIT2000 multicenter clinical trial cohort. Acta Neuropathol 2014; 128:137–149.

Rizzo D, Ruggiero A, Martini M, et al. Molecular biology in pediatric high-grade glioma: impact on prognosis and treatment. Biomed Res Int 2015; 2015:215135.

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Last update: 18 September 2017