Skip to main content
Log in

Challenges in the management of childhood low-grade glioma in a developing country

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

Abstract

Background

Treatment modality impacts outcome of childhood low-grade glioma (LGG). Optimizing management in developing countries can be challenging. This study evaluates the clinical characteristics, treatment, and factors influencing outcome of childhood LGG in Saudi Arabia.

Patients and methods

This study retrospectively evaluated 59 children consecutively diagnosed with LGG between January 2001 and June 2016.

Results

Median age at diagnosis was 6.0 years. Pilocytic astrocytoma represented 64.9% of cases. The anatomic site was cerebellar in 23.7%, cerebral in 18.6%, hypothalamic-optic pathway in 33.9%, and midline in 23.7%. The 5-year overall survival (OS) and progression-free survival (PFS) were 90.6 ± 4.7 and 54.3 ± 8.4%, respectively. Initial treatment was observation in 28.8%, surgery alone in 35.6%, chemotherapy in 13.6%, radiotherapy in 5.1%, and combined in 16.9% of cases. The corresponding 5-year PFS was 56.3 ± 15.6, 53.3 ± 14.0, 22.9 ± 19.7, 33.3 ± 27.2, and 88.9 ± 10.5%, respectively (p = 0.006). Among the 61% who had surgical intervention (either alone or in combination with other therapies), 22% achieved complete resection with 5-year radiation/progression-free survival (RPFS) of 87.5 ± 11.7% compared to 27.6 ± 10.8% for subtotal resection/biopsy and 62.2 ± 17.0% for no surgery (p = 0.013). Adjuvant therapy for residual tumor improved survival with 5-year PFS of 66.7 ± 19.2% for chemotherapy and 100% for radiotherapy compared to 12.5 ± 11.4% for observation (p = 0.033).

Conclusions

We identified variability in the outcomes of LGG. Fewer surgeries with lower rates of total resection were noted, compared to reports from international cooperative groups. The extent of resection was predictive of RPFS. Adjuvant therapy improved the outcome of patients with residual disease, resulting in PFS rates comparable to international data.

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

Access this article

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

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

References

  1. Louis DN, Ohagaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) WHO classification of tumours of the central nervous system, 4th edn. IARC press, Lyon

    Google Scholar 

  2. Bukhard C, Di Patre PL, Schüler D, Schüler G, Yaşargil MG, Yonekawa Y, Lütolf UM, Kleihues P, Ohgaki H (2003) A population based study of the incidence and survival rates in pateints with pilocytic astrocytoma. J Neurosurg 98(6):1170–1174. https://doi.org/10.3171/jns.2003.98.6.1170

    Article  Google Scholar 

  3. Listernick R, Ferner RE, Liu GT, Gutmann DH (2007) Optic pathway glioma in neurofibromatosis-1: controversies and recommendations. Ann Neurol 61(3):189–198. https://doi.org/10.1002/ana.21107

    Article  CAS  PubMed  Google Scholar 

  4. Schmandt SM, Packer RJ (2000) Treatment of low-grade pediatric gliomas. Curr Opin Oncol 12(3):194–198. https://doi.org/10.1097/00001622-200005000-00002

    Article  CAS  PubMed  Google Scholar 

  5. Due-Tonnessen BJ, Helseth E, Scheie D, Skullerud K, Aamodt G, Lundar T (2002) Long term outcome after resection of benign cerebellar astrocytomas in children and young adults (0-19 years): report of 110 consecutive cases. Pediatr Neurosurg 37(2):71–80. https://doi.org/10.1159/000065108

    Article  PubMed  Google Scholar 

  6. Wisoff JH, Sanford RA, Heier LA, Sposto R, Burger PC, Yates AJ, Holmes EJ, Kun LE (2006) Primary neurosurgery for pediatric low grade gliomas: a prospective multi-institutional study from the children’s oncology group. Neurosurgery 42(6):347–353

    Google Scholar 

  7. Gnekow AK, Falkenstein F, von Hornstein S et al (2012) Long-term follow-up of the multicenter, multidisciplinary treatment study HIT-LGG-1996 for low-grade glioma in children and adolescents of the German Speaking Society of Pediatric Oncology and Hematology. Neuro-Oncology 14(10):1265–1284

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Stockland T, Liu JF, Ironside JW, Ellison DW, Taylor R, Robinson KJ, Picton SV, Walker DA (2010) A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702). Neuro-Oncology 12(12):1257–1268

    Google Scholar 

  9. Bouffet E, Jakacki R, Goldman S et al (2012) Phase II study of weekly vinblastine in recurrent or refractory pediatric low-grade glioma. J Clin Oncol 30:1358–1363

    Article  CAS  PubMed  Google Scholar 

  10. Lassaletta A, Scheinemann K, Zelcer SM et al (2016) Phase II weekly vinblastine for chemotherapy-naive children with progressive low-grade glioma: a Canadian pediatric brain tumor consortium study. J Clin Oncol 34:3537–3543

    Article  CAS  PubMed  Google Scholar 

  11. Ater J, Holmes E, Zhou T et al (2008) Results of the COG protocol 9952: a randomized phase 3 study of 2 chemotherapy regimens for incompletely resected low grade glioma in young children. Neuro-Oncology 10:452

    Google Scholar 

  12. Packer RJ, Meadows AT, Rocke LB, Goldwein JL, D'Angio G (1987) Long-term sequelae of cancer treatment on the central nervous system in childhood. Med Pediatr Oncol 15(5):241–253. https://doi.org/10.1002/mpo.2950150505

    Article  CAS  PubMed  Google Scholar 

  13. Doireau V, Grill J, Zerah M, Lellouch-Tubiana A, Couanet D, P Chastagner P, Marchal JC, Grignon Y, Chouffai Z and Kalifa C (1999) For the Brain Tumours Subcommittee of the French Society of Paediatric Oncology (SFOP). Chemotherapy for unresectable and recurrent intramedullary glial tumours in children. Br J Cancer 81(5):835–840

  14. Waanders AJ, Minturn JE, Fisher MJ Pediatric low-grade gliomas: diagnosis, treatment, and future firections. In: Hayat MA (ed) Tumors of the central nervous system, vol 14. https://doi.org/10.1007/978-94-017-7224-2_2

  15. Fisher PG, Tihan T, Goldthwaite PT, Wharam MD, Carson BS, Weingart JD, Repka MX, Cohen KJ, Burger PC (2008) Outcome analysis of childhood low-grade astrocytomas. Pediatr Blood Cancer 51(2):245–250. https://doi.org/10.1002/pbc.21563

    Article  PubMed  Google Scholar 

  16. Pollack IF, Claassen D, al-Shboul Q, Janosky JE, Deutsch M (1995) Low-grade gliomas of the cerebral hemispheres in children: an analysis of 71 cases. J Neurosurg 82(4):536–547. https://doi.org/10.3171/jns.1995.82.4.0536

    Article  CAS  PubMed  Google Scholar 

  17. Desai KI, Nadkarni TD, Muzumdar DP, Goel A (2001) Prognostic factors for cerebellar astrocytomas in children: a study of 102 cases. Pediatr Neurosurg 35(6):311–317. https://doi.org/10.1159/000050443

    Article  CAS  PubMed  Google Scholar 

  18. Opochera E, Kremer LC, Da Daltb L, van de Weteringa MD, Viscardi E, Caron HN, HN PG (2006) Prognostic factors for progression of childhood optic pathway glioma: a systematic review. Eur J Cancer 42(12):1807–1816. https://doi.org/10.1016/j.ejca.2006.02.022

    Article  Google Scholar 

  19. Taylor T, Jaspan T, Milano G, Gregson R, Parker T, Ritzmann T, Benson C, Walker D on behalf of the PLAN study group (2008) Radiological classification of optic pathway gliomas: experience of a modified functional classification system. Br J Radiol 81:761–766, 970, DOI: https://doi.org/10.1259/bjr/65246351

  20. Gnekow AK (1995) Recommendations of the Brain Tumor Subcommittee for the reporting of trials. SIOP Brain Tumor Subcommittee International Society of Pediatric Oncology. Med Pediatr Oncol 24(2):104–108

    Article  CAS  PubMed  Google Scholar 

  21. Bandopadhayay P, Bergthold G, London WB et al (2014) Long-term outcome of 4,040 children diagnosed with pediatric low-grade gliomas: an analysis of the Surveillance Epidemiology and End Results (SEER) database. Pediatr Blood Cancer 61(7):1173–1179

    Article  PubMed  PubMed Central  Google Scholar 

  22. Puget S, Crimmins DC, Garnett MR, Grill J, Oliveira R, Boddaert N, Wray A, Lelouch-Tubiana A, Roujeau T, Di Rocco F, Zerah M, Sainte-Rose C (2007) Thalamic tumors in children: a reappraisal. J Neurosurg 106(5 Suppl Pediatrics):354–362

    PubMed  Google Scholar 

  23. Reardon DA, Gajjar A, Sanford RA, Heideman RL, Walter AW, Thompson SJ, Merchant TE, Li H, Jenkins JJ, Langston J, Boyett JM, Kun LE (1998) Bithalamic involvement predicts poor outcome among children with thalamic glial tumors. Pedaitr Neurosurg 29(1):29–35. https://doi.org/10.1159/000028681

    Article  CAS  Google Scholar 

  24. Al-Jumaily UA (2015) Outcome of pediatric low grade glioma in developing countries: the impact of elimination of radiotherapy. Karbala J Med 8(1):2131–2140

    Google Scholar 

  25. Karim AB, Afra D, Cornu P, Bleehan N, Schraub S, De-Witte O, Darcel F, Stenning S, Pierart M, Van-Glabbeke M (2002) Randomized trial on the efficacy of radiotherapy for cerebral low-grade glioma in the adult: European Organization for Research and Treatment of Cancer Study 22845 with the Medical Research Council Study BRO4: an interim analysis. Int J Radiat Oncol Biol Phys 52(2):316–324. https://doi.org/10.1016/S0360-3016(01)02692-X

    Article  PubMed  Google Scholar 

  26. Baumert BG, Stupp R (2010) Is there a place for radiotherapy in low-grade gliomas? Adv Tech Stand Neurosurg 35:159–182

    Article  CAS  PubMed  Google Scholar 

  27. Kortmann R-D, Timmermann B, Taylor RE, Scarzello G, Plasswilm L, Paulsen F, Jeremic B, Gnekow AK, Dieckmann K, Kay S, Bamberg M (2003) Current and future strategies in radiotherapy of childhood low-grade glioma of the brain part I: treatment modalities of radiation therapy. Strahlenther Onkol 179(8):509–520. https://doi.org/10.1007/s00066-003-9104-9

    Article  PubMed  Google Scholar 

  28. Packer RJ, Sutton LN, Atkins TE, Radcliffe J, Bunin GR, D'Angio G, Siegel KR, Schut L (1989) A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. J Neurosurg 70(5):707–713. https://doi.org/10.3171/jns.1989.70.5.0707

    Article  CAS  PubMed  Google Scholar 

  29. Johnson DL, McCabe MA, Nicholson HS, Joseph AL, Getson PR, Byrne J, Brasseux C, Packer RJ, Reaman G (1994) Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80(6):1004–1010. https://doi.org/10.3171/jns.1994.80.6.1004

    Article  CAS  PubMed  Google Scholar 

  30. Scheinemann K, Bartels U, Tsangaris E, Hawkins C, Huang A, Dirks P, Fried I, Bouffet E, Tabori U (2011) Feasibility and efficacy of repeated chemotherapy for progressive pediatric low-grade gliomas. Pediatr Blood Cancer 57:84–88

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Khalid Abdalla.

Ethics declarations

Conflict of interest

The authors have no conflicts of interest to disclose.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Abdalla, K., Abdullah, S., Almehdar, A. et al. Challenges in the management of childhood low-grade glioma in a developing country. Childs Nerv Syst 34, 861–870 (2018). https://doi.org/10.1007/s00381-018-3729-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-018-3729-6

Keywords

Navigation