Abstract
Since tumours of the central nervous system (CNS) are rare in children to begin with, there are certain types that are very rare, occurring with a frequency of less than 5 % of all paediatric CNS tumours. These deserve special focus because of some key challenges. For one, the initial descriptive histology gathered from a small series of tumour samples makes it challenging to define unique characteristics for some very rare tumours. Most pathologists and clinicians will encounter examples of these tumours infrequently, adding a challenge to their recognition and the overall awareness of a management strategy. From a clinical perspective, tumours that are ‘very rare’ cannot be studied in a specific randomized trial in order to determine the best course of treatment; therefore the treating physician must rely upon case series and reports, always paying special attention to the exception to the rules. In this chapter, we outline some key examples of very rare paediatric CNS tumours and discuss how to approach some of these challenges.
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Dolecek TA, Propp JM, Stroup NE, Kruchko C. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2005–2009. Neuro Oncol. 2012;14 Suppl 5:v1–49.
Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:97–109.
Villani A, Tabori U, Schiffman J, et al. Biochemical and imaging surveillance in germline TP53 mutation carriers with Li–Fraumeni syndrome: a prospective observational study. Lancet Oncol. 2011;12:559–67.
Lafay-Cousin L, Keene D, Carret AS, et al. Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience. Childs Nerv Syst. 2011;27: 259–64.
Lafay-Cousin L, Mabbott DJ, Halliday W, et al. Use of ifosfamide, carboplatin, and etoposide chemotherapy in choroid plexus carcinoma. J Neurosurg Pediatr. 2010;5:615–21.
Wrede B, Liu P, Wolff JE. Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol. 2007;85:345–51.
Kane LA, Leinung MC, Scheithauer BW, et al. Pituitary adenomas in childhood and adolescence. J Clin Endocrinol Metab. 1994;79:1135–40.
Harrington MH, Casella SJ. Pituitary tumors in childhood. Curr Opin Endocrinol Diabetes Obes. 2012; 19:63–7.
Schlechte JA. Clinical practice. Prolactinoma. N Engl J Med. 2003;349:2035–41.
Zagzag D, Blanco C, Friedlander DR, Miller DC, Newcomb EW. Expression of p27KIP1 in human gliomas: relationship between tumor grade, proliferation index, and patient survival. Hum Pathol. 2003;34:48–53.
Kantar M, Ertan Y, Turhan T, et al. Anaplastic astroblastoma of childhood: aggressive behavior. Childs Nerv Syst. 2009;25:1125–9.
Fevre-Montange M, Hasselblatt M, Figarella-Branger D, et al. Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. J Neuropathol Exp Neurol. 2006;65:1004–11.
Lu-Emerson C, Plotkin SR. The neurofibromatoses. Part 2: NF2 and schwannomatosis. Rev Neurol Dis. 2009;6:E81–6.
Propp JM, McCarthy BJ, Davis FG, Preston-Martin S. Descriptive epidemiology of vestibular schwannomas. Neuro Oncol. 2006;8:1–11.
Plotkin SR, Stemmer-Rachamimov AO, Barker 2nd FG, et al. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 2009;361:358–67.
Ozlen F, Gunduz A, Asan Z, et al. Dysembryoplastic neuroepithelial tumors and gangliogliomas: clinical results of 52 patients. Acta Neurochir. 2010;152: 1661–71.
Daghistani R, Miller E, Kulkarni AV, Widjaja E. Atypical characteristics and behavior of dysembryoplastic neuroepithelial tumors. Neuroradiology. 2013;55:217–24.
Schindler G, Capper D, Meyer J, et al. Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma. Acta Neuropathol. 2011;121:397–405.
Koelsche C, Wohrer A, Jeibmann A, et al. Mutant BRAF V600E protein in ganglioglioma is predominantly expressed by neuronal tumor cells. Acta Neuropathol. 2013;125:891–900.
Dahiya S, Haydon DH, Alvarado D, Gurnett CA, Gutmann DH, Leonard JR. BRAF(V600E) mutation is a negative prognosticator in pediatric ganglioglioma. Acta Neuropathol. 2013;125:901–10.
Maiuri F, Spaziante R, De Caro ML, Cappabianca P, Giamundo A, Iaconetta G. Central neurocytoma: clinico-pathological study of 5 cases and review of the literature. Clin Neurol Neurosurg. 1995;97: 219–28.
Ibrahim GM, Fallah A, Shahideh M, Tabori U, Rutka JT. Primary Ewing’s sarcoma affecting the central nervous system: a review and proposed prognostic considerations. J Clin Neurosci. 2012;19:203–9.
Chi SN, Zimmerman MA, Yao X, et al. Intensive multimodality treatment for children with newly diagnosed CNS atypical teratoid rhabdoid tumor. J Clin Oncol. 2009;27:385–9.
Fleming AJ, Hukin J, Rassekh R, et al. Atypical teratoid rhabdoid tumors (ATRTs): the British Columbia’s Children’s Hospital’s experience, 1986–2006. Brain Pathol. 2012;22:625–35.
Jones DT, Jager N, Kool M, et al. Dissecting the genomic complexity underlying medulloblastoma. Nature. 2012;488:100–5.
Schwartzentruber J, Korshunov A, Liu XY, et al. Driver mutations in histone H3.3 and chromatin remodelling genes in paediatric glioblastoma. Nature. 2012;482:226–31.
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Fleming, A.J. (2015). Rare Tumours of the Central Nervous System in Children. In: Scheinemann, K., Bouffet, E. (eds) Pediatric Neuro-oncology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1541-5_17
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DOI: https://doi.org/10.1007/978-1-4939-1541-5_17
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