SpringerLink
Log in

Brainstem Tumors in Children

  • Chapter
  • First Online:
Oncology of CNS Tumors

  • 1263 Accesses

Abstract

Brainstem tumors are a disparate group of tumors that span the regions of the midbrain to the cervicomedullary junction (Fig. 20.1). They most frequently occur in children and lack a specific gender predilection. Over the past two decades, we have become increasingly more familiar with their characteristic patient symptom complex, their specific neuroimaging features, and a standardized approach to therapy. That said, there are still a number of challenges that are posed by tumors in this critical region of the neuraxis. As each tumor subtype could be the subject of an independent chapter, here, we will review the main patient presentations, the key neuroimaging features, and the goals of surgical and non-operative therapies for children with brainstem tumors.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
EUR 29.95
Price includes VAT (Germany)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
EUR 160.49
Price includes VAT (Germany)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
EUR 213.99
Price includes VAT (Germany)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free ship** worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Igboechi C, Vaddiparti A, Sorenson EP, Rozzelle CJ, Tubbs RS, Loukas M (2013) Tectal plate gliomas: a review. Childs Nerv Syst 29:1827–1833

    Article  Google Scholar 

  2. Diaz RJ, Girgis FM, Hamiltonn MG (2014) Endoscopic third ventriculostomy for hydrocephalus due to tectal glioma. Can J Neurol Sci 41:476–481

    Article  Google Scholar 

  3. Chumas PD, Drake JM, Del Bigio MR (1992) Death from chronic tonsillar herniation in a patient with lumboperitoneal shunt and Crouzon’s disease. Br J Neurosurg 6:595–599

    Article  CAS  Google Scholar 

  4. Vandertop WP, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, Amstrong DC, Becker LE (1992) Focal midbrain tumors in children. Neurosurgery 31:186–194

    Article  CAS  Google Scholar 

  5. Broadway SJ, Ogg RJ, Scoggins MA, Sanford R, Patay Z, Boop FA (2011) Surgical management of tumors producing the thalamopeduncular syndrome of childhood. J Neurosurg Pediatr 7:589–595

    Article  Google Scholar 

  6. Lee RP, Foster KA, Lillard JC, Klimo P Jr, Ellison DW, Orr B, Boop FA (2017) Surgical and molecular considerations in the treatment of pediatric thalamopeduncular tumors. J Neurosurg Pediatr 20:247–255

    Article  Google Scholar 

  7. Ng WH, Mukhida K, Rutka JT (2010) Image guidance and neuromonitoring in neurosurgery. Childs Nerv Syst 26:491–502

    Article  Google Scholar 

  8. Packer RJ, Pfister S, Bouffet E, Avery R, Bandopadhayay P, Bornhorst M, Bowers DC, Ellison D, Fangusaro J, Foreman N, Fouladi M, Gajjar A, Haas-Kogan D, Hawkins C, Ho CY, Hwang E, Jabado N, Kilburn LB, Lassaletta A, Ligon KL, Massimino M, Meeteren SV, Mueller S, Nicolaides T, Perilongo G, Tabori U, Vezina G, Warren K, Witt O, Zhu Y, Jones DT, Kieran M (2017) Pediatric low-grade gliomas: implications of the biologic era. Neuro Oncol 19:750–761

    CAS  PubMed  Google Scholar 

  9. Mathew RK, Rutka JT (2018) Diffuse intrinsic pontine glioma: clinical features, molecular genetics, and novel targeted therapeutics. J Korean Neurosurg Soc 61:343–351

    Article  CAS  Google Scholar 

  10. Buczkowicz P, Hoeman C, Rakopoulos P, Pajovic S, Letourneau L, Dzamba M, Morrison A, Lewis P, Bouffet E, Bartels U, Zuccaro J, Agnihotri S, Ryall S, Barszczyk M, Chornenkyy Y, Bourgey M, Bourque G, Montpetit A, Cordero F, Castelo-Branco P, Mangerel J, Tabori U, Ho KC, Huang A, Taylor KR, Mackay A, Bendel AE, Nazarian J, Fangusaro JR, Karajannis MA, Zagzag D, Foreman NK, Donson A, Hegert JV, Smith A, Chan J, Lafay-Cousin L, Dunn S, Hukin J, Dunham C, Scheinemann K, Michaud J, Zelcer S, Ramsay D, Cain J, Brennan C, Souweidane MM, Jones C, Allis CD, Brudno M, Becher O, Hawkins C (2014) Genomic analysis of diffuse intrinsic pontine gliomas identifies three molecular subgroups and recurrent activating ACVR1 mutations. Nat Genet 46:451–456

    Article  CAS  Google Scholar 

  11. Fontebasso AM, Papillon-Cavanagh S, Schwartzentruber J, Nikbakht H, Gerges N, Fiset PO, Bechet D, Faury D, De Jay N, Ramkissoon LA, Corcoran A, Jones DT, Sturm D, Johann P, Tomita T, Goldman S, Nagib M, Bendel A, Goumnerova L, Bowers DC, Leonard JR, Rubin JB, Alden T, Browd S, Geyer JR, Leary S, Jallo G, Cohen K, Gupta N, Prados MD, Carret AS, Ellezam B, Crevier L, Klekner A, Bognar L, Hauser P, Garami M, Myseros J, Dong Z, Siegel PM, Malkin H, Ligon AH, Albrecht S, Pfister SM, Ligon KL, Majewski J, Jabado N, Kieran MW (2014) Recurrent somatic mutations in ACVR1 in pediatric midline high-grade astrocytoma. Nat Genet 46:462–466

    Article  CAS  Google Scholar 

  12. Hoffman LM, Veldhuijzen van Zanten SEM, Colditz N, Baugh J, Chaney B, Hoffmann M, Lane A, Fuller C, Miles L, Hawkins C, Bartels U, Bouffet E, Goldman S, Leary S, Foreman NK, Packer R, Warren KE, Broniscer A, Kieran MW, Minturn J, Comito M, Broxson E, Shih CS, Khatua S, Chintagumpala M, Carret AS, Escorza NY, Hassall T, Ziegler DS, Gottardo N, Dholaria H, Doughman R, Benesch M, Drissi R, Nazarian J, Jabado N, Boddaert N, Varlet P, Giraud G, Castel D, Puget S, Jones C, Hulleman E, Modena P, Giagnacovo M, Antonelli M, Pietsch T, Gielen GH, Jones DTW, Sturm D, Pfister SM, Gerber NU, Grotzer MA, Pfaff E, von Bueren AO, Hargrave D, Solanki GA, Jadrijevic Cvrlje F, Kaspers GJL, Vandertop WP, Grill J, Bailey S, Biassoni V, Massimino M, Calmon R, Sanchez E, Bison B, Warmuth-Metz M, Leach J, Jones B, van Vuurden DG, Kramm CM, Fouladi M (2018) Clinical, radiologic, pathologic, and molecular characteristics of long-term survivors of diffuse intrinsic pontine glioma (DIPG): a collaborative report from the International and European Society for Pediatric Oncology DIPG registries. J Clin Oncol 36:1963–1972

    Article  CAS  Google Scholar 

  13. Truffaux N, Philippe C, Paulsson J, Andreiuolo F, Guerrini-Rousseau L, Cornilleau G, Le Dret L, Richon C, Lacroix L, Puget S, Geoerger B, Vassal G, Ostman A, Grill J (2015) Preclinical evaluation of dasatinib alone and in combination with cabozantinib for the treatment of diffuse intrinsic pontine glioma. Neuro Oncol 17:953–964

    Article  CAS  Google Scholar 

  14. Puget S, Beccaria K, Blauwblomme T, Roujeau T, James S, Grill J, Zerah M, Varlet P, Sainte-Rose C (2015) Biopsy in a series of 130 pediatric diffuse intrinsic pontine gliomas. Childs Nerv Syst 31:1773–1780

    Article  Google Scholar 

  15. Puget, S., Blauwblomme, T., and Grill, J. (2012) Is biopsy safe in children with newly diagnosed diffuse intrinsic pontine glioma? Am Soc Clin Oncol Educ Book 629–633

    Google Scholar 

  16. Plessier A, Le Dret L, Varlet P, Beccaria K, Lacombe J, Meriaux S, Geffroy F, Fiette L, Flamant P, Chretien F, Blauwblomme T, Puget S, Grill J, Debily MA, Castel D (2017) New in vivo avatars of diffuse intrinsic pontine gliomas (DIPG) from stereotactic biopsies performed at diagnosis. Oncotarget 8:52543–52559

    Article  Google Scholar 

  17. Souweidane MM, Kramer K, Pandit-Taskar N, Zhou Z, Haque S, Zanzonico P, Carrasquillo JA, Lyashchenko SK, Thakur SB, Donzelli M, Turner RS, Lewis JS, Cheung NV, Larson SM, Dunkel IJ (2018) Convection-enhanced delivery for diffuse intrinsic pontine glioma: a single-centre, dose-escalation, phase 1 trial. Lancet Oncol 19:1040–1050

    Article  Google Scholar 

  18. Zhou Z, Singh R, Souweidane MM (2017) Convection-enhanced delivery for diffuse intrinsic pontine glioma treatment. Curr Neuropharmacol 15:116–128

    Article  CAS  Google Scholar 

  19. Luther N, Zhou Z, Zanzonico P, Cheung NK, Humm J, Edgar MA, Souweidane MM (2014) The potential of theragnostic (1)(2)(4)I-8H9 convection-enhanced delivery in diffuse intrinsic pontine glioma. Neuro Oncol 16:800–806

    Article  CAS  Google Scholar 

  20. Zhou Z, Luther N, Ibrahim GM, Hawkins C, Vibhakar R, Handler MH, Souweidane MM (2013) B7-H3, a potential therapeutic target, is expressed in diffuse intrinsic pontine glioma. J Neurooncol 111:257–264

    Article  CAS  Google Scholar 

  21. Benitez-Ribas D, Cabezon R, Florez-Grau G, Molero MC, Puerta P, Guillen A, Paco S, Carcaboso AM, Santa-Maria Lopez V, Cruz O, de Torres C, Salvador N, Juan M, Mora J, La Madrid AM (2018) Immune response generated with the administration of autologous dendritic cells pulsed with an allogenic tumoral cell-lines lysate in patients with newly diagnosed diffuse intrinsic pontine glioma. Front Oncol 8:127

    Article  Google Scholar 

  22. Alli S, Figueiredo CA, Golbourn B, Sabha N, Wu MY, Bondoc A, Luck A, Coluccia D, Maslink C, Smith C, Wurdak H, Hynynen K, O’Reilly M, Rutka JT (2018) Brainstem blood brain barrier disruption using focused ultrasound: a demonstration of feasibility and enhanced doxorubicin delivery. J Control Release 281:29–41

    Article  CAS  Google Scholar 

  23. Pollack IF, Hoffman HJ, Humphreys RP, Becker L (1993) The long-term outcome after surgical treatment of dorsally exophytic brain-stem gliomas. J Neurosurg 78:859–863

    Article  CAS  Google Scholar 

  24. Stone SS, Rutka JT (2008) Utility of neuronavigation and neuromonitoring in epilepsy surgery. Neurosurg Focus 25:E17

    Article  Google Scholar 

  25. Timoney N, Rutka JT (2017) Recent advances in epilepsy surgery and achieving best outcomes using high-frequency oscillations, diffusion tensor imaging, magnetoencephalography, intraoperative neuromonitoring, focal cortical dysplasia, and bottom of sulcus dysplasia. Neurosurgery 64:1–10

    Article  Google Scholar 

  26. Green AL, Kieran MW (2015) Pediatric brainstem gliomas: new understanding leads to potential new treatments for two very different tumors. Curr Oncol Rep 17:436

    Article  Google Scholar 

  27. Lassaletta A, Zapotocky M, Mistry M, Ramaswamy V, Honnorat M, Krishnatry R, Guerreiro Stucklin A, Zhukova N, Arnoldo A, Ryall S, Ling C, McKeown T, Loukides J, Cruz O, de Torres C, Ho CY, Packer RJ, Tatevossian R, Qaddoumi I, Harreld JH, Dalton JD, Mulcahy-Levy J, Foreman N, Karajannis MA, Wang S, Snuderl M, Nageswara Rao A, Giannini C, Kieran M, Ligon KL, Garre ML, Nozza P, Mascelli S, Raso A, Mueller S, Nicolaides T, Silva K, Perbet R, Vasiljevic A, Faure Conter C, Frappaz D, Leary S, Crane C, Chan A, Ng HK, Shi ZF, Mao Y, Finch E, Eisenstat D, Wilson B, Carret AS, Hauser P, Sumerauer D, Krskova L, Larouche V, Fleming A, Zelcer S, Jabado N, Rutka JT, Dirks P, Taylor MD, Chen S, Bartels U, Huang A, Ellison DW, Bouffet E, Hawkins C, Tabori U (2017) Therapeutic and prognostic implications of BRAF V600E in pediatric low-grade gliomas. J Clin Oncol 35:2934–2941

    Article  CAS  Google Scholar 

  28. Penman CL, Faulkner C, Lowis SP, Kurian KM (2015) Current understanding of BRAF alterations in diagnosis, prognosis, and therapeutic targeting in pediatric low-grade gliomas. Front Oncol 5:54

    Article  Google Scholar 

  29. McAbee JH, Modica J, Thompson CJ, Broniscer A, Orr B, Choudhri AF, Boop FA, Klimo P Jr (2015) Cervicomedullary tumors in children. J Neurosurg Pediatr 16:357–366

    Article  Google Scholar 

  30. Janjua MB, Ivasyk I, Pisapia DJ, Souweidane MM (2017) Ganglioglioma of brain stem and cervicomedullary junction: a 50 years review of literature. J Clin Neurosci 44:34–46

    Article  Google Scholar 

  31. Garcia MA, Solomon DA, Haas-Kogan DA (2016) Exploiting molecular biology for diagnosis and targeted management of pediatric low-grade gliomas. Future Oncol 12:1493–1506

    Article  CAS  Google Scholar 

  32. Sturm D, Pfister SM, Jones DTW (2017) Pediatric gliomas: current concepts on diagnosis, biology, and clinical management. J Clin Oncol 35:2370–2377

    Article  Google Scholar 

Download references

Acknowledgments

This chapter was made possible through funds provided from the Canadian Institutes of Health Research, Brainchild, the Wiley Family Foundation, and the Laurie Berman Fund at the Hospital for Sick Children.

Author information

Authors and Affiliations

  1. Division of Neurosurgery, The Hospital for Sick Children, Toronto, ON, Canada

    Ali S. Haider & James M. Drake

  2. Division of Neurosurgery, The Arthur and Sonia Labatt Brain Tumour Research Centre, The Hospital for Sick Children, Toronto, ON, Canada

    James T. Rutka

  3. Department of Surgery, University of Toronto, Toronto, ON, Canada

    James T. Rutka

Authors
  1. Ali S. Haider
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James M. Drake
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. James T. Rutka
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to James T. Rutka .

Editor information

Editors and Affiliations

  1. Department of Neurosurgery, University of Munich Department of Neurosurgery, Munich, Germany

    Jörg-Christian Tonn

  2. Dana-Farber Cancer Institute Center, Harvard University, Boston, USA

    David A. Reardon

  3. SickKids Hospital, University of Toronto, Toronto, ON, Canada

    James T. Rutka

  4. Department of Neurosurgery, University of Hamburg, Hamburg, Germany

    Manfred Westphal

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Haider, A.S., Drake, J.M., Rutka, J.T. (2019). Brainstem Tumors in Children. In: Tonn, JC., Reardon, D., Rutka, J., Westphal, M. (eds) Oncology of CNS Tumors. Springer, Cham. https://doi.org/10.1007/978-3-030-04152-6_20

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-04152-6_20

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-04151-9

  • Online ISBN: 978-3-030-04152-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation