Skip to main content
SpringerLink
Log in

Long-term visual outcomes of optic pathway gliomas in pediatric patients without neurofibromatosis type 1

  • Clinical Study
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Sporadic optic pathway gliomas (OPGs) have been reported to cause more vision loss than OPGs associated with neurofibromatosis type-1, but long-term visual outcome data are limited. The purpose of this study was to report the visual outcomes of a cohort of pediatric patients with sporadic OPGs. This was a retrospective, cohort study at a tertiary care pediatric hospital and cancer institute. The study included all patients with sporadic OPGs evaluated from 1990 to 2014. The primary outcome was visual acuity at final follow-up. Secondary outcomes were risk factors for a poor visual outcome and the rate of progression. There were 59 pediatric patients included in the study. Median age at presentation was 2.5 years old and median follow-up was 5.2 years. In the worse eye at final follow-up, 16 patients (27 %) were 20/30 or better, 9 patients (15 %) were between 20/40 and 20/80, and 34 patients (58 %) were 20/100 or worse. In the better eye at final follow-up, 33 patients (56 %) were 20/30 or better, 11 patients (19 %) were between 20/40 and 20/80, and 15 patients (25 %) were 20/100 or worse. Risk factors for a poor visual outcome included younger age at presentation, optic nerve pallor, and tumor extent. Of the 54 patients (92 %) who received treatment, 40 (74 %) experienced disease progression during or after treatment. A majority of pediatric patients with sporadic OPGs had significant long-term visual impairment. In spite of treatment, tumor progression is common. Serial ophthalmic examinations with quantitative vision measurements are essential in the management of sporadic OPGs.

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

Similar content being viewed by others

References

  1. Dolecek TA, Propp JM, Stroup NE, Kruchko C (2012) CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2005–2009. Neuro-oncology 14(Suppl 5):v1–v49

    Article  PubMed  PubMed Central  Google Scholar 

  2. Avery RA, Ferner RE, Listernick R, Fisher MJ, Gutmann DH, Liu GT (2012) Visual acuity in children with low grade gliomas of the visual pathway: implications for patient care and clinical research. J Neuro-oncol 110(1):1–7

    Article  Google Scholar 

  3. Fisher MJ, Avery RA, Allen JC, Ardern-Holmes SL, Bilaniuk LT, Ferner RE, Gutmann DH, Listernick R, Martin S, Ullrich NJ, Liu GT (2013) Functional outcome measures for NF1-associated optic pathway glioma clinical trials. Neurology 81(21 Suppl 1):S15–S24

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Singhal S, Birch JM, Kerr B, Lashford L, Evans DG (2002) Neurofibromatosis type 1 and sporadic optic gliomas. Arch Dis Child 87(1):65–70

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Czyzyk E, Jóźwiak S, Roszkowski M, Schwartz RA (2003) Optic pathway gliomas in children with and without neurofibromatosis 1. J Child Neurol 18(7):471–478

    Article  PubMed  Google Scholar 

  6. Astrup J (2003) Natural history and clinical management of optic pathway glioma. Br J Neurosurg 17(4):327–335

    Article  CAS  PubMed  Google Scholar 

  7. Pediatric Eye Disease Investigator Group (2002) A randomized trial of atropine vs. patching for treatment of moderate amblyopia in children. Arch Ophthalmol 120(3):268–278

    Article  Google Scholar 

  8. StataCorp (2013) Stata Statistical Software: Release 13

  9. Dunn OJ (1959) Estimation of the medians for dependent variables. Ann Math Stat 30(1):192–197

    Article  Google Scholar 

  10. Dunn OJ (1964) Multiple comparisons using rank sums. Technometrics 6(3):241–252

    Article  Google Scholar 

  11. Campagna M, Opocher E, Viscardi E, Calderone M, Severino SM, Cermakova I, Perilongo G (2010) Optic pathway glioma: long-term visual outcome in children without neurofibromatosis type-1. Pediatr Blood Cancer 55(6):1083–1088

    Article  PubMed  Google Scholar 

  12. Willard VW, Conklin HM, Wu S, Merchant TE (2015) Prospective longitudinal evaluation of emotional and behavioral functioning in pediatric patients with low-grade glioma treated with conformal radiation therapy. J Neurooncol 122(1):161–168

    Article  PubMed  PubMed Central  Google Scholar 

  13. Thiagalingam S, Flaherty M, Billson F, North K (2004) Neurofibromatosis type 1 and optic pathway gliomas: follow-up of 54 patients. Ophthalmology 111(3):568–577

    Article  PubMed  Google Scholar 

  14. Balcer LJ, Liu GT, Heller G, Bilaniuk L, Volpe NJ, Galetta SL, Molloy PT, Phillips PC, Janss AJ, Vaughn S, Maguire MG (2001) Visual loss in children with neurofibromatosis type 1 and optic pathway gliomas: relation to tumor location by magnetic resonance imaging. Am J Ophthalmol 131(4):442–445

    Article  CAS  PubMed  Google Scholar 

  15. Segal L, Darvish-Zargar M, Dilenge ME, Ortenberg J, Polomeno RC (2010) Optic pathway gliomas in patients with neurofibromatosis type 1: follow-up of 44 patients. J AAPOS 14(2):155–158

    Article  PubMed  Google Scholar 

  16. Fisher MJ, Loguidice M, Gutmann DH, Listernick R, Ferner RE, Ullrich NJ, Packer RJ, Tabori U, Hoffman RO, Ardern-Holmes SL, Hummel TR, Hargrave DR, Bouffet E, Charrow J, Bilaniuk LT, Balcer LJ, Liu GT (2012) Visual outcomes in children with neurofibromatosis type 1-associated optic pathway glioma following chemotherapy: a multicenter retrospective analysis. Neuro-oncology 14(6):790–797

    Article  PubMed  PubMed Central  Google Scholar 

  17. Tow SL, Chandela S, Miller NR, Avellino AM (2003) Long-term outcome in children with gliomas of the anterior visual pathway. Pediatr Neurol 28(4):262–270

    Article  PubMed  Google Scholar 

  18. Liu GT, Brodsky MC, Phillips PC, Belasco J, Janss A, Golden JC, Bilaniuk LL, Burson GT, Duhaime AC, Sutton LN (2004) Optic radiation involvement in optic pathway gliomas in neurofibromatosis. Am J Ophthalmol 137(3):407–414

    Article  PubMed  Google Scholar 

  19. Chan MY, Foong AP, Heisey DM, Harkness W, Hayward R, Michalski A (1998) Potential prognostic factors of relapse-free survival in childhood optic pathway glioma: a multivariate analysis. Pediatr Neurosurg 29(1):23–28

    Article  CAS  PubMed  Google Scholar 

  20. Nicolin G, Parkin P, Mabbott D, Hargrave D, Bartels U, Tabori U, Rutka J, Buncic JR, Bouffet E (2009) Natural history and outcome of optic pathway gliomas in children. Pediatr Blood Cancer 53(7):1231–1237

    Article  PubMed  Google Scholar 

  21. Laithier V, Grill J, Le Deley MC, Ruchoux MM, Couanet D, Doz F, Pichon F, Rubie H, Frappaz D, Vannier JP, Babin-Boilletot A, Sariban E, Chastagner P, Zerah M, Raguin MA, Hartmann O, Kalifa C (2003) Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy—results of the first French prospective study for the French Society of Pediatric Oncology. J Clin Oncol 21(24):4572–4578

    Article  CAS  PubMed  Google Scholar 

  22. Grill J, Laithier V, Rodriguez D, Raquin MA, Pierre-Kahn A, Kalifa C (2000) When do children with optic pathway tumours need treatment? An oncological perspective in 106 patients treated in a single centre. Eur J Pediatr 159(9):692–696

    Article  CAS  PubMed  Google Scholar 

  23. Opocher E, Kremer LC, Da Dalt L, van de Wetering MD, Viscardi E, Caron HN, Perilongo G (2006) Prognostic factors for progression of childhood optic pathway glioma: a systematic review. Eur J Cancer 42(12):1807–1816

    Article  PubMed  Google Scholar 

  24. Kelly JP, Weiss AH (2013) Detection of tumor progression in optic pathway glioma with and without neurofibromatosis type 1. Neuro-oncology 15(11):1560–1567

    Article  PubMed  PubMed Central  Google Scholar 

  25. Kalin-Hajdu E, Décarie J-C, Marzouki M, Carret A-S, Ospina LH (2014) Visual acuity of children treated with chemotherapy for optic pathway gliomas. Pediatr Blood Cancer 61(2):223–227

    Article  CAS  PubMed  Google Scholar 

  26. Mitchell AE, Elder JE, Mackey DA, Waters KD, Ashley DM (2001) Visual improvement despite radiologically stable disease after treatment with carboplatin in children with progressive low-grade optic/thalamic gliomas. J Pediatr Hematol Oncol 23(9):572–577

    Article  CAS  PubMed  Google Scholar 

  27. Parsa CF (2012) Why visual function does not correlate with optic glioma size or growth. Arch Ophthalmol 130(4):521–522

    Article  PubMed  Google Scholar 

  28. Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM, Muci-Mendoza R, Ramella M, Manor RS, Fletcher WA, Repka MX, Garrity JA, Ebner RN, Monteiro ML, McFadzean RM, Rubtsova IV, Hoyt WF (2001) Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging. Arch Ophthalmol 119(4):516–529

    Article  CAS  PubMed  Google Scholar 

  29. Thomas RP, Gibbs IC, Xu LW, Recht L (2015) Treatment options for optic pathway gliomas. Curr Treat Options Neurol 17(2):333

    Article  PubMed  Google Scholar 

  30. Packer RJ, Ater J, Allen J, Phillips P, Geyer R, Nicholson HS, Jakacki R, Kurczynski E, Needle M, Finlay J, Reaman G, Boyett JM (1997) Carboplatin and vincristine chemotherapy for children with newly diagnosed progressive low-grade gliomas. J Neurosurg 86(5):747–754

    Article  CAS  PubMed  Google Scholar 

  31. Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S (2010) Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence. Eur J Cancer 46(12):2253–2259

    Article  PubMed  Google Scholar 

  32. Gutmann DH, Donahoe J, Brown T, James CD, Perry A (2000) Loss of neurofibromatosis 1 (NF1) gene expression in NF1-associated pilocytic astrocytomas. Neuropathol Appl Neurobiol 26(4):361–367

    Article  CAS  PubMed  Google Scholar 

  33. Jones DT, Kocialkowski S, Liu L, Pearson DM, Bäcklund LM, Ichimura K, Collins VP (2008) Tandem duplication producing a novel oncogenic BRAF fusion gene defines the majority of pilocytic astrocytomas. Cancer Res 68(21):8673–8677

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Ophthalmology, Boston Children’s Hospital, Harvard Medical School, 300 Longwood Avenue, Fegan 4, Boston, MA, 02115, USA

    Michael J. Wan & Gena Heidary

  2. Department of Neurology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, 02115, USA

    Nicole J. Ullrich

  3. Department of Hematology/Oncology, Boston Children’s Hospital, Harvard Medical School, Boston, MA, 02115, USA

    Nicole J. Ullrich, Peter E. Manley & Mark W. Kieran

  4. Pediatric Neuro-Oncology, Dana-Farber/Boston Children’s Hospital Center for Cancer and Blood Disorders, Boston, MA, 02115, USA

    Nicole J. Ullrich, Peter E. Manley & Mark W. Kieran

  5. Department of Neurosurgery, Boston Children’s Hospital, Harvard Medical School, Boston, MA, 02115, USA

    Liliana C. Goumnerova

Authors
  1. Michael J. Wan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Nicole J. Ullrich
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Peter E. Manley
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Mark W. Kieran
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Liliana C. Goumnerova
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Gena Heidary
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gena Heidary.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedure performed in studies involving human participants were in accordance with the ethical standards of Boston Children’s Hospital and Harvard University and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wan, M.J., Ullrich, N.J., Manley, P.E. et al. Long-term visual outcomes of optic pathway gliomas in pediatric patients without neurofibromatosis type 1. J Neurooncol 129, 173–178 (2016). https://doi.org/10.1007/s11060-016-2163-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11060-016-2163-4

Keywords

Search

Navigation