Abstract
Diffuse intrinsic pontine glioma (DIPG) is a devastating childhood malignancy. The tumors are diagnosed radiographically and symptomatically. Biopsy is not recommended. The pathology is almost always a WHO grade II, III, or IV astrocytoma. Patients present with less than 6 months of cranial nerve defects, ataxia, and/or long tract signs. Radiographically, tumors are hypointense on T1, hyperintense on T2 and have an infiltrating mass that occupies at least 2/3 of the pons. The standard of care for treatment of a DIPG is radiation therapy. Neither surgery nor chemotherapy has been found to improve survival for these children. The average survival from diagnosis is 8–12 months.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Badhe PB, Chauhan PP, Mehta NK (2004) Brainstem gliomas–a clinicopathological study of 45 cases with p53 immunohistochemistry. Indian J Cancer 41(4):170–174
Benesch M, Wagner S, Berthold F, Wolff JE (2005) Primary dissemination of high-grade gliomas in children: experiences from four studies of the Pediatric Oncology and Hematology Society of the German Language Group (GPOH). J Neurooncol 72(2):179–183
Broniscer A, Gajjar A (2004) Supratentorial high-grade astrocytoma and diffuse brainstem glioma: two challenges for the pediatric oncologist. Oncologist 9(2):197–206
Broniscer A, Laningham FH, Sanders RP, Kun LE, Ellison DW et al (2008) Young age may predict a better outcome for children with diffuse pontine glioma. Cancer 113(3):566–572
Broniscer A, Baker JN, Tagen M, Onar-Thomas A, Gilbertson RJ et al (2010) Phase I study of vandetanib during and after radiotherapy in children with diffuse intrinsic pontine glioma. J Clin Oncol 28(31):4762–4768
Cartmill M, Punt J (1999) Diffuse brain stem glioma. A review of stereotactic biopsies. Childs Nerv Syst 15(5):235–237; discussion 238
Cohen KJ, Heideman RL, Zhou T, Holmes EJ, Lavey RS et al (2011) Temozolomide in the treatment of children with newly diagnosed diffuse intrinsic pontine gliomas: a report from the Children’s Oncology Group. Neuro Oncol 13(4):410–416
Curless RG, Bowen BC, Pattany PM, Gonik R, Kramer DL (2002) Magnetic resonance spectroscopy in childhood brainstem tumors. Pediatr Neurol 26(5):374–378
Donaldson SS, Laningham F, Fisher PG (2006) Advances toward an understanding of brainstem gliomas. J Clin Oncol 24(8):1266–1272
Dreyer ZE, Kadota RP, Stewart CF, Friedman HS, Mahoney DH et al (2003) Phase 2 study of idarubicin in pediatric brain tumors: Pediatric Oncology Group study POG 9237. Neuro Oncol 5(4):261–267
Grill J, Puget S, Andreiuolo F, Philippe C, MacConaill L et al (2012) Critical oncogenic mutations in newly diagnosed pediatric diffuse intrinsic pontine glioma. Pediatr Blood Cancer 58(4):489–491
Guillamo JS, Monjour A, Taillandier L, Devaux B, Varlet P et al (2001) Brainstem gliomas in adults: prognostic factors and classification. Brain 124(Pt 12):2528–2539
Gururangan S, Chi SN, Young Poussaint T, Onar-Thomas A, Gilbertson RJ et al (2010) Lack of efficacy of bevacizumab plus irinotecan in children with recurrent malignant glioma and diffuse brainstem glioma: a Pediatric Brain Tumor Consortium study. J Clin Oncol 28(18):3069–3075
Haas-Kogan DA, Banerjee A, Poussaint TY, Kocak M, Prados MD et al (2011) Phase II trial of tipifarnib and radiation in children with newly diagnosed diffuse intrinsic pontine gliomas. Neuro Oncol 13(3):298–306
Hendriksen EM, Span PN, Schuuring J, Peters JP, Sweep FC et al (2009) Angiogenesis, hypoxia and VEGF expression during tumour growth in a human xenograft tumour model. Microvasc Res 77(2):96–103
Hibi T, Shitara N, Genka S, Fuchinoue T, Hayakawa I et al (1992) Radiotherapy for pediatric brain stem glioma: radiation dose, response, and survival. Neurosurgery 31(4):643–650; discussion 650–641
Jallo G (2006) Brainstem gliomas. Childs Nerv Syst 22(1):1–2
Jallo GI, Biser-Rohrbaugh A, Freed D (2004) Brainstem gliomas. Childs Nerv Syst 20(3):143–153
Kesari S, Kim RS, Markos V, Drappatz J, Wen PY et al (2008) Prognostic factors in adult brainstem gliomas: a multicenter, retrospective analysis of 101 cases. J Neurooncol 88(2):175–183
Knauer-Fischer S, Schaper J, Janssen HT, Ratjen F (1999) Tuberculoma of the pons. Pediatr Neurol 20(1):57–59
Korones DN (2007) Treatment of newly diagnosed diffuse brain stem gliomas in children: in search of the holy grail. Expert Rev Anticancer Ther 7(5):663–674
Laprie A, Pirzkall A, Haas-Kogan DA, Cha S, Banerjee A et al (2005) Longitudinal multivoxel MR spectroscopy study of pediatric diffuse brainstem gliomas treated with radiotherapy. Int J Radiat Oncol Biol Phys 62(1):20–31
Lashford LS, Thiesse P, Jouvet A, Jaspan T, Couanet D et al (2002) Temozolomide in malignant gliomas of childhood: a United Kingdom Children’s Cancer Study Group and French Society for Pediatric Oncology Intergroup Study. J Clin Oncol 20(24):4684–4691
Lewis J, Lucraft H, Gholkar A (1997) UKCCSG study of accelerated radiotherapy for pediatric brain stem gliomas. United Kingdom Childhood Cancer Study Group. Int J Radiat Oncol Biol Phys 38(5):925–929
Liu AK, Macy ME, Foreman NK (2009) Bevacizumab as therapy for radiation necrosis in four children with pontine gliomas. Int J Radiat Oncol Biol Phys 75(4):1148–1154
MacDonald TJ (2012) Diffuse intrinsic pontine glioma (DIPG): time to biopsy again? Pediatr Blood Cancer 58(4):487–488
Massimino M, Spreafico F, Biassoni V, Simonetti F, Riva D et al (2008) Diffuse pontine gliomas in children: changing strategies, changing results? A mono-institutional 20-year experience. J Neurooncol 87(3):355–361
Packer RJ, Prados M, Phillips P, Nicholson HS, Boyett JM et al (1996) Treatment of children with newly diagnosed brain stem gliomas with intravenous recombinant beta-interferon and hyperfractionated radiation therapy: a childrens cancer group phase I/II study. Cancer 77(10):2150–2156
Paugh BS, Broniscer A, Qu C, Miller CP, Zhang J et al (2011) Genome-wide analyses identify recurrent amplifications of receptor tyrosine kinases and cell-cycle regulatory genes in diffuse intrinsic pontine glioma. J Clin Oncol 29(30):3999–4006
Pierre-Kahn A, Hirsch JF, Vinchon M, Payan C, Sainte-Rose C et al (1993) Surgical management of brain-stem tumors in children: results and statistical analysis of 75 cases. J Neurosurg 79(6):845–852
Pollack IF, Jakacki RI, Blaney SM, Hancock ML, Kieran MW et al (2007) Phase I trial of imatinib in children with newly diagnosed brainstem and recurrent malignant gliomas: a Pediatric Brain Tumor Consortium report. Neuro Oncol 9(2):145–160
Pollack IF, Stewart CF, Kocak M, Poussaint TY, Broniscer A et al (2011) A phase II study of gefitinib and irradiation in children with newly diagnosed brainstem gliomas: a report from the Pediatric Brain Tumor Consortium. Neuro Oncol 13(3):290–297
Sheline GE (1975) Radiation therapy of tumors of the central nervous system in childhood. Cancer 35(3 suppl):957–964
Shuper A, Kornreich L, Loven D, Michowitz S, Schwartz M et al (1998) Diffuse brain stem gliomas. Are we improving outcome? Childs Nerv Syst 14(10):578–581
Szerlip NJ, Pedraza A, Chakravarty D, Azim M, McGuire J et al (2012) Intratumoral heterogeneity of receptor tyrosine kinases EGFR and PDGFRA amplification in glioblastoma defines subpopulations with distinct growth factor response. Proc Natl Acad Sci U S A 109(8):3041–3046
Thakur SB, Karimi S, Dunkel IJ, Koutcher JA, Huang W (2006) Longitudinal MR spectroscopic imaging of pediatric diffuse pontine tumors to assess tumor aggression and progression. AJNR Am J Neuroradiol 27(4):806–809
Wagner S, Benesch M, Berthold F, Gnekow AK, Rutkowski S et al (2006) Secondary dissemination in children with high-grade malignant gliomas and diffuse intrinsic pontine gliomas. Br J Cancer 95(8):991–997
Warren K, Bent R, Wolters PL, Prager A, Hanson R et al (2012) A phase 2 study of pegylated interferon alpha-2b (PEG-Intron((R))) in children with diffuse intrinsic pontine glioma. Cancer 118(14):3607–3613
Wolff JE, Driever PH, Erdlenbruch B, Kortmann RD, Rutkowski S et al (2010) Intensive chemotherapy improves survival in pediatric high-grade glioma after gross total resection: results of the HIT-GBM-C protocol. Cancer 116(3):705–712
Zarghooni M, Bartels U, Lee E, Buczkowicz P, Morrison A et al (2010) Whole-genome profiling of pediatric diffuse intrinsic pontine gliomas highlights platelet-derived growth factor receptor alpha and poly (ADP-ribose) polymerase as potential therapeutic targets. J Clin Oncol 28(8):1337–1344
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media Dordrecht
About this chapter
Cite this chapter
Bredlau, A.L., Korones, D.N. (2014). Diffuse Intrinsic Pontine Gliomas in Children: Treatment (An Update). In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 11. Tumors of the Central Nervous System, vol 11. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-7037-9_6
Download citation
DOI: https://doi.org/10.1007/978-94-007-7037-9_6
Published:
Publisher Name: Springer, Dordrecht
Print ISBN: 978-94-007-7036-2
Online ISBN: 978-94-007-7037-9
eBook Packages: MedicineMedicine (R0)