Skip to main content

An Overview of Pediatric High-Grade Gliomas and Diffuse Intrinsic Pontine Gliomas

  • Chapter
  • First Online:
Pediatric Cancer, Volume 2

Part of the book series: Pediatric Cancer ((PECA,volume 2))

  • 1408 Accesses

Abstract

High-grade gliomas represent approximately 10% of all pediatric brain tumors. Diffuse intrinsic pontine gliomas are a distinct clinical entity in pediatrics and accounts for another 10% of central nervous system tumors in children. Pediatric patients with high-grade gliomas or diffuse intrinsic pontine gliomas have a very poor prognosis despite a variety of aggressive therapies, including surgery, chemotherapy and radiation therapy. Though these tumors behave similarly to adult high-grade gliomas, there are important differences in the molecular biology of high-grade gliomas between children and adults. In this chapter, we present an overview of both pediatric high-grade gliomas as well as diffuse intrinsic pontine gliomas. We focus upon their epidemiology, etiology, clinical presentation and biologic features. In addition, we discuss treatment approaches, prognostic factors, outcomes and future research directions.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Bondy ML, Scheurer ME, Malmer B, Barnholtz-Sloan JS, Davis FG, Il’yasova D, Kruchko C, McCarthy BJ, Rajaraman P, Schwartzbaum JA, Sadetzki S, Schlehofer B, Tihan T, Wiemels JL, Wrensch M, Buffler PA (2008) Brain tumor epidemiology: consensus from the brain tumor epidemiology consortium. Cancer 113:1953–1968

    Article  PubMed  Google Scholar 

  • Bredel M, Pollack IF, Hamilton RL, James CD (1999) Epidermal growth factor receptor expression and gene amplification in high-grade non-brainstem gliomas of childhood. Clin Cancer Res 5:1786–1792

    PubMed  CAS  Google Scholar 

  • Broniscer A, Iacono L, Chintagumpala M, Fouladi M, Wallace D, Bowers DC, Stewart C, Krasin MJ, Gajjar A (2005) Role of temozolomide after radiotherapy for newly diagnosed diffuse brainstem glioma in children: results of a multiinstitutional study (SJHG-98). Cancer 103:133–139

    Article  PubMed  CAS  Google Scholar 

  • Broniscer A, Chintagumpala M, Fouladi M, Krasin MJ, Kocak M, Bowers DC, Iacono LC, Merchant TE, Stewart CF, Houghton PJ, Kun LE, Ledet D, Gajjar A (2006) Temozolomide after radiotherapy for newly diagnosed high-grade glioma and unfavorable low-grade glioma in children. J Neurooncol 76:313–319

    Article  PubMed  Google Scholar 

  • Buckner JC, Brown PD, O’Neill BP, Meyer FB, Wetmore CJ, Uhm JH (2007) Central nervous system tumors. Mayo Clin Proc 82:1271–1286

    Article  PubMed  Google Scholar 

  • Cartmill M, Punt J (1999) Diffuse brain stem glioma. A review of stereotactic biopsies. Childs Nerv Syst 15:235–237, discussion 238

    Article  PubMed  CAS  Google Scholar 

  • CBTRUS (2005) Statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2004–2005. Central Brain Tumor Registry of the United States. Hinsdale, IL, www.cbtrus.org

  • Chen W (2007) Clinical applications of PET in brain tumors. J Nucl Med 48:1468–1481

    Article  PubMed  Google Scholar 

  • Choux M, Lena G, Do L (2000) Brainstem tumors. In: Choux M, DiRocco C, Hockley A (eds) Pedaitric neurosurgery. Churchill Livingstone, New York, pp 471–491

    Google Scholar 

  • Duffner PK, Horowitz ME, Krischer JP, Friedman HS, Burger PC, Cohen ME, Sanford RA, Mulhern RK, James HE, Freeman CR (1993) Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328:1725–1731

    Article  PubMed  CAS  Google Scholar 

  • Fangusaro J (2009) Pediatric high-grade gliomas and diffuse intrinsic pontine gliomas. J Child Neurol 24:1409–1417

    Article  PubMed  Google Scholar 

  • Finlay JL, Boyett JM, Yates AJ, Wisoff JH, Milstein JM, Geyer JR, Bertolone SJ, McGuire P, Cherlow JM, Tefft M (1995) Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimen. Childrens Cancer Group. J Clin Oncol 13:112–123

    PubMed  CAS  Google Scholar 

  • Finlay JL, Dhall G, Boyett JM, Dunkel IJ, Gardner SL, Goldman S, Yates AJ, Rosenblum MK, Stanley P, Zimmerman RA, Wallace D, Pollack IF, Packer RJ (2008) Myeloablative chemotherapy with autologous bone marrow rescue in children and adolescents with recurrent malignant astrocytoma: outcome compared with conventional chemotherapy: a report from the Children’s Oncology Group. Pediatr Blood Cancer 51:806–811

    Article  PubMed  Google Scholar 

  • Frazier JL, Lee J, Thomale UW, Noggle JC, Cohen KJ, Jallo GI (2009) Treatment of diffuse intrinsic brainstem gliomas: failed approaches and future strategies. J Neurosurg Pediatr 3:259–269

    Article  PubMed  Google Scholar 

  • Gilbertson RJ, Hill DA, Hernan R, Kocak M, Geyer R, Olson J, Gajjar A, Rush L, Hamilton RL, Finkelstein SD, Pollack IF (2003) ERBB1 is amplified and ­overexpressed in high-grade diffusely infiltrative pediatric brain stem glioma. Clin Cancer Res 9:3620–3624

    PubMed  CAS  Google Scholar 

  • Glantz MJ, Cole BF, Forsyth PA, Recht LD, Wen PY, Chamberlain MC, Grossman SA, Cairncross JG (2000) Practice parameter: anticonvulsant prophylaxis in patients with newly diagnosed brain tumors. Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 54:1886–1893

    PubMed  CAS  Google Scholar 

  • Gururangan S, Chi S, Onar A, Packer R, Poussaint T, Gilbertson R, Friedman H, Kun L, Boyett J (2008) Abstracts for the thirteenth annual meeting of the Society for Neuro-Oncology: Phase II study of bevacizumab plus irinotecan in children with recurrent malignant glioma and diffuse brainstem glioma – a Pediatric Brain Tumor Consortium Study (PBTC-022). Neuro-oncology 10:759–915

    Article  Google Scholar 

  • Hargrave D, Bartels U, Bouffet E (2006) Diffuse brainstem glioma in children: critical review of clinical trials. Lancet Oncol 7:241–248

    Article  PubMed  Google Scholar 

  • Herrington B, Kieran MW (2009) Small molecule inhibitors in children with malignant gliomas. Pediatr Blood Cancer 53:312–317

    Article  PubMed  Google Scholar 

  • Jenkin RD, Boesel C, Ertel I, Evans A, Hittle R, Ortega J, Sposto R, Wara W, Wilson C, Anderson J (1987) Brain-stem tumors in childhood: a prospective randomized trial of irradiation with and without adjuvant CCNU, VCR, and prednisone. A report of the Childrens Cancer Study Group. J Neurosurg 66:227–233

    Article  PubMed  CAS  Google Scholar 

  • Jennings MT, Sposto R, Boyett JM, Vezina LG, Holmes E, Berger MS, Bruggers CS, Bruner JM, Chan KW, Dusenbery KE, Ettinger LJ, Fitz CR, Lafond D, Mandelbaum DE, Massey V, McGuire W, McNeely L, Moulton T, Pollack IF, Shen V (2002) Preradiation chemotherapy in primary high-risk brainstem tumors: phase II study CCG-9941 of the Children’s Cancer Group. J Clin Oncol 20:3431–3437

    Article  PubMed  CAS  Google Scholar 

  • Lemort M, Canizares-Perez AC, Van der Stappen A, Kampouridis S (2007) Progress in magnetic resonance imaging of brain tumours. Curr Opin Oncol 19:616–622

    Article  PubMed  Google Scholar 

  • Lonser RR, Warren KE, Butman JA, Quezado Z, Robison RA, Walbridge S, Schiffman R, Merrill M, Walker ML, Park DM, Croteau D, Brady RO, Oldfield EH (2007) Real-time image-guided direct convective perfusion of intrinsic brainstem lesions. Technical note. J Neurosurg 107:190–197

    Article  PubMed  Google Scholar 

  • Louis DN, Rubio MP, Correa KM, Gusella JF, von Deimling A (1993) Molecular genetics of pediatric brain stem gliomas. Application of PCR techniques to small and archival brain tumor specimens. J Neuropathol Exp Neurol 52:507–515

    Article  PubMed  CAS  Google Scholar 

  • Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109

    Article  PubMed  Google Scholar 

  • Melean G, Sestini R, Ammannati F, Papi L (2004) Genetic insights into familial tumors of the nervous system. Am J Med Genet C Semin Med Genet 129C:74–84

    Article  PubMed  Google Scholar 

  • Nakamura M, Shimada K, Ishida E, Higuchi T, Nakase H, Sakaki T, Konishi N (2007) Molecular pathogenesis of pediatric astrocytic tumors. Neuro-oncology 9:113–123

    Article  PubMed  CAS  Google Scholar 

  • Neglia JP, Robison LL, Stovall M, Liu Y, Packer RJ, Hammond S, Yasui Y, Kasper CE, Mertens AC, Donaldson SS, Meadows AT, Inskip PD (2006) New primary neoplasms of the central nervous system in survivors of childhood cancer: a report from the Childhood Cancer Survivor Study. J Natl Cancer Inst 98:1528–1537

    Article  PubMed  Google Scholar 

  • Pollack IF, Finkelstein SD, Woods J, Burnham J, Holmes EJ, Hamilton RL, Yates AJ, Boyett JM, Finlay JL, Sposto R (2002) Expression of p53 and prognosis in children with malignant gliomas. N Engl J Med 346:420–427

    Article  PubMed  CAS  Google Scholar 

  • Pollack IF, Hamilton RL, Sobol RW, Burnham J, Yates AJ, Holmes EJ, Zhou T, Finlay JL (2006) O6-methylguanine-DNA methyltransferase expression strongly correlates with outcome in childhood malignant gliomas: results from the CCG-945 Cohort. J Clin Oncol 24:3431–3437

    Article  PubMed  CAS  Google Scholar 

  • Rickert CH, Strater R, Kaatsch P, Wassmann H, Jurgens H, Dockhorn-Dworniczak B, Paulus W (2001) Pediatric high-grade astrocytomas show chromosomal imbalances distinct from adult cases. Am J Pathol 158:1525–1532

    Article  PubMed  CAS  Google Scholar 

  • Rood BR, MacDonald TJ (2005) Pediatric high-grade glioma: molecular genetic clues for innovative therapeutic approaches. J Neurooncol 75:267–272

    Article  PubMed  CAS  Google Scholar 

  • Rosenfeld A, Listernick R, Charrow J, Goldman S (2010) Neurofibromatosis type 1 and high-grade tumors of the central nervous system. Childs Nerv Syst 26(5):663–667

    Article  PubMed  Google Scholar 

  • Sanders RP, Kocak M, Burger PC, Merchant TE, Gajjar A, Broniscer A (2007) High-grade astrocytoma in very young children. Pediatr Blood Cancer 49:888–893

    Article  PubMed  Google Scholar 

  • Sposto R, Ertel IJ, Jenkin RD, Boesel CP, Venes JL, Ortega JA, Evans AE, Wara W, Hammond D (1989) The effectiveness of chemotherapy for treatment of high grade astrocytoma in children: results of a randomized trial. A report from the Childrens Cancer Study Group. J Neurooncol 7:165–177

    Article  PubMed  CAS  Google Scholar 

  • Stupp R, Mason WP, van den Bent MJ, Weller M, Fisher B, Taphoorn MJ, Belanger K, Brandes AA, Marosi C, Bogdahn U, Curschmann J, Janzer RC, Ludwin SK, Gorlia T, Allgeier A, Lacombe D, Cairncross JG, Eisenhauer E, Mirimanoff RO (2005) Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med 352:987–996

    Article  PubMed  CAS  Google Scholar 

  • Wagner S, Warmuth-Metz M, Emser A, Gnekow AK, Strater R, Rutkowski S, Jorch N, Schmid HJ, Berthold F, Graf N, Kortmann RD, Pietsch T, Sorensen N, Peters O, Wolff JE (2006) Treatment options in childhood pontine gliomas. J Neurooncol 79:281–287

    Article  PubMed  Google Scholar 

  • Walter AW, Hancock ML, Pui CH, Hudson MM, Ochs JS, Rivera GK, Pratt CB, Boyett JM, Kun LE (1998) Secondary brain tumors in children treated for acute lymphoblastic leukemia at St Jude Children’s Research Hospital. J Clin Oncol 16:3761–3767

    PubMed  CAS  Google Scholar 

  • Ward BA, Gutmann DH (2005) Neurofibromatosis 1: from lab bench to clinic. Pediatr Neurol 32:221–228

    Article  PubMed  Google Scholar 

  • Warren KE (2008) Pediatric high-grade gliomas 2008 educational book. Am Soc Clin Oncol:472477

    Google Scholar 

  • Wisoff JH, Boyett JM, Berger MS, Brant C, Li H, Yates AJ, McGuire-Cullen P, Turski PA, Sutton LN, Allen JC, Packer RJ, Finlay JL (1998) Current neurosurgical management and the impact of the extent of resection in the treatment of malignant gliomas of childhood: a report of the Children’s Cancer Group trial no. CCG-945. J Neurosurg 89:52–59

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Rishi R. Lulla .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer Science+Business Media Dordrecht

About this chapter

Cite this chapter

Lulla, R.R., Fangusaro, J. (2012). An Overview of Pediatric High-Grade Gliomas and Diffuse Intrinsic Pontine Gliomas. In: Hayat, M. (eds) Pediatric Cancer, Volume 2. Pediatric Cancer, vol 2. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-2957-5_26

Download citation

Publish with us

Policies and ethics