Skip to main content
SpringerLink
Log in

Diffuse intrinsic pontine glioma in children and adolescents: a single-center experience

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Background

Patients with diffuse intrinsic pontine glioma (DIPG) have a very poor prognosis. Only radiotherapy (XRT) has proven to be effective in delaying the disease progression. Several chemotherapy schedules have been applied so far, but none demonstrated significant improvements in progression and survival.

Methods

We retrospectively analyzed the clinical data of children diagnosed with DIPG at our center (Pediatric Hospital “Regina Margherita,” Turin, Italy) between 1999 and 2013. Progression-free survival (PFS) and overall survival (OS) were used to describe the outcomes.

Results

Twenty-four children were included in our report. Patients diagnosed before March 2003 (n = 12) were treated with XRT and vincristine (VCR); the remaining 12 patients received XRT and temozolomide (TMZ). Progression-free survival was 18.8 % at 1 year (SE = 7.6 %), while overall survival was 44.1 % at 1 year (SE = 9.9 %). Median PFS was 8.1 months, whereas median OS was 11.2 months. No statistically significant difference in PFS or OS was evidenced between the two treatment groups.

Conclusion

Radiotherapy followed by VCR or TMZ allows obtaining results that are in line with previous reports, with no advantages over other similar treatment schedules.

DIPGs are challenging tumors with a dismal outcome. Further research and newer therapies are urgently needed in order to achieve improvements in survival.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. Albright AL, Packer RJ, Zimmerman R, Rorke LB, Boyett J, Hammond GD (1993) Magnetic resonance scans should replace biopsies for the diagnosis of diffuse brain stem gliomas: a report from the Children’s Cancer Group. [Miscellaneous Article]. Neurosurg 33:1026–1030

    Article  CAS  Google Scholar 

  2. Bailey S, Howman A, Wheatley K, Wherton D, Boota N, Pizer B, Fisher D, Kearns P, Picton S, Saran F, Gibson M, Glaser A, Connolly DJA, Hargrave D (n.d.) Diffuse intrinsic pontine glioma treated with prolonged temozolomide and radiotherapy—Results of a United Kingdom phase II trial (CNS 2007 04). Eur. J. Cancer. Available: http://www.sciencedirect.com/science/article/pii/S0959804913007703. Accessed 13 Nov 2013

  3. Bouffet E, Raquin M, Doz F, Gentet J-C, Rodary C, Demeocq F, Chastagner P, Lutz P, Hartmann O, Kalifa C (2000) Radiotherapy followed by high-dose busulfan and thiotepa. Cancer 88:685–692. doi:10.1002/(SICI)1097-0142(20000201)88:3<685::AID-CNCR27>3.0.CO;2-K

    Article  CAS  PubMed  Google Scholar 

  4. 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. Cancer 103:133–139. doi:10.1002/cncr.20741

    Article  CAS  PubMed  Google Scholar 

  5. Cage TA, Samagh SP, Mueller S, Nicolaides T, Haas-Kogan D, Prados M, Banerjee A, Auguste KI, Gupta N (2013) Feasibility, safety, and indications for surgical biopsy of intrinsic brainstem tumors in children. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 29:1313–1319. doi:10.1007/s00381-013-2101-0

    Article  Google Scholar 

  6. Cohen KJ, Heideman RL, Zhou T, Holmes EJ, Lavey RS, Bouffet E, Pollack IF (2011) Temozolomide in the treatment of children with newly diagnosed diffuse intrinsic pontine gliomas: a report from the Children’s Oncology Group. Neuro-Oncol 13:410–416. doi:10.1093/neuonc/noq205

    Article  PubMed Central  PubMed  Google Scholar 

  7. Fontanilla HP, Pinnix CC, Ketonen LM, Woo SY, Vats TS, Rytting ME, Wolff JE, Mahajan A (2012) Palliative re-irradiation for progressive diffuse intrinsic pontine glioma. Am J Clin Oncol 35:51–57

    Article  PubMed  Google Scholar 

  8. Frappaz D, Schell M, Thiesse P, Marec-Bérard P, Mottolese C, Perol D, Bergeron C, Philip T, Ricci AC, Galand-Desme S, Szathmari A, Carrie C (2008) Preradiation chemotherapy may improve survival in pediatric diffuse intrinsic brainstem gliomas: final results of BSG 98 prospective trial. Neuro-Oncol 10:599–607. doi:10.1215/15228517-2008-029

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  9. Freeman CR, Suissa S (1986) Brain stem tumors in children: results of a survey of 62 patients treated with radiotherapy. Int J Radiat Oncol 12:1823–1828. doi:10.1016/0360-3016(86)90325-1

    Article  CAS  Google Scholar 

  10. Garzón M, García-Fructuoso G, Guillén A, Suñol M, Mora J, Cruz O (2013) Brain stem tumors in children and adolescents: single institutional experience. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 29:1321–1331. doi:10.1007/s00381-013-2137-1

    Article  Google Scholar 

  11. Jansen MHA, Van Vuurden DG, Vandertop WP, Kaspers GJL (2012) Diffuse intrinsic pontine gliomas: a systematic update on clinical trials and biology. Cancer Treat Rev 38:27–35

    Article  CAS  PubMed  Google Scholar 

  12. 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. doi:10.3171/jns.1987.66.2.0227

    Article  CAS  PubMed  Google Scholar 

  13. Kaplan E, Meier P (1958) Nonparametric estimation from incomplete estimations. J Am Stat Assoc 457–481

  14. Kebudi R, Cakir FB, Agaoglu FY, Gorgun O, Ayan I, Darendeliler E (2013) Pediatric diffuse intrinsic pontine glioma patients from a single center. Childs Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg 29:583–588. doi:10.1007/s00381-012-1986-3

    Article  Google Scholar 

  15. Korones DN, Fisher PG, Kretschmar C, Zhou T, Chen Z, Kepner J, Freeman C (2008) Treatment of children with diffuse intrinsic brain stem glioma with radiotherapy, vincristine, and oral VP-16: a Children’s Oncology Group phase II study. Pediatr Blood Cancer 50:227–230. doi:10.1002/pbc.21154

    Article  PubMed  Google Scholar 

  16. Lewis J, Lucraft H, Gholkar A (1997) UKCCSG study of accelerated radiotherapy for pediatric brain stem gliomas. Int J Radiat Oncol 38:925–929. doi:10.1016/S0360-3016(97)00134-X

    Article  CAS  Google Scholar 

  17. MacDonald TJ (2012) Diffuse intrinsic pontine glioma (DIPG): time to biopsy again? Pediatr Blood Cancer 58:487–488. doi:10.1002/pbc.24090

    Article  PubMed  Google Scholar 

  18. Mandell LR, Kadota R, Freeman C, Douglass EC, Fontanesi J, Cohen ME, Kovnar E, Burger P, Sanford RA, Kepner J, Friedman H, Kun LE (1999) There is no role for hyperfractionated radiotherapy in the management of children with newly diagnosed diffuse intrinsic brainstem tumors: results of a Pediatric Oncology Group phase III trial comparing conventional versus hyperfractionated radiotherapy. Int J Radiat Oncol Biol Phys 43:959–964

    Article  CAS  PubMed  Google Scholar 

  19. Massimino M, Spreafico F, Biassoni V, Simonetti F, Riva D, Trecate G, Giombini S, Poggi G, Pecori E, Pignoli E (2008) Diffuse pontine gliomas in children: changing strategies, changing results? A mono-institutional 20-year experience. J Neurooncol 87:355–361

    Article  CAS  PubMed  Google Scholar 

  20. Packer RJ, Boyett JM, Zimmerman RA, Rorke LB, Kaplan AM, Albright AL, Selch MT, Finlay JL, Hammond GD, Wara WM (1993) Hyperfractionated radiation therapy (72 Gy) for children with brain stem gliomas: a Childrens Cancer Group phase I/II trial. Cancer 72:1414–1421. doi:10.1002/1097-0142(19930815)72:4<1414::AID-CNCR2820720442>3.0.CO;2-C

    Article  CAS  PubMed  Google Scholar 

  21. Peto R, Peto J (1972) Asymptotically efficient rank invariant test procedures. J R Stat Soc Ser Gen 135:185–207. doi:10.2307/2344317

    Article  Google Scholar 

  22. Qaddoumi I, Sultan I, Gajjar A (2009) Outcome and prognostic features in pediatric gliomas. Cancer 115:5761–5770. doi:10.1002/cncr.24663

    Article  PubMed Central  PubMed  Google Scholar 

  23. Walker DA, Liu J, Kieran M, Jabado N, Picton S, Packer R, St Rose C, Paris CPN (2011) Conference Consensus Group (2013): a multi-disciplinary consensus statement concerning surgical approaches to low-grade, high-grade astrocytomas, and diffuse intrinsic pontine gliomas in childhood (CPN Paris 2011) using the Delphi method. Neuro-Oncol 15:462–468. doi:10.1093/neuonc/nos330

    Article  Google Scholar 

Download references

Acknowledgments

Vallero SG has been funded by The Italian Cord Blood Donors Association (ADISCO).

None of the Authors has financial conflicts of interest. We declare that this manuscript is original, has not been published before, and is not currently being considered for publication elsewhere. The manuscript has been read and approved by all named authors.

The study has been performed in accordance with the 1964 Declaration of Helsinki and its later amendments. All patients, or their parents when needed, gave their informed consent prior to their inclusion in the study.

Author information

Authors and Affiliations

  1. Pediatric Oncohematology, Stem Cell Transplantation and Cell Therapy Division, A.O. Città della Salute e della Scienza—Ospedale Infantile “Regina Margherita”, Piazza Polonia, 94, 10126, Turin, Italy

    Stefano Gabriele Vallero, Daniele Bertin, Maria Eleonora Basso, Laura Stefania Pittana & Franca Fagioli

  2. Radiotherapy Unit, A.O. Città della Salute e della Scienza—Ospedale Infantile “Regina Margherita”, Turin, Italy

    Anna Mussano

Authors
  1. Stefano Gabriele Vallero
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Daniele Bertin
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Maria Eleonora Basso
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Laura Stefania Pittana
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Anna Mussano
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Franca Fagioli
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Stefano Gabriele Vallero.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Vallero, S.G., Bertin, D., Basso, M.E. et al. Diffuse intrinsic pontine glioma in children and adolescents: a single-center experience. Childs Nerv Syst 30, 1061–1066 (2014). https://doi.org/10.1007/s00381-014-2359-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-014-2359-x

Keywords

Search

Navigation