Child's Nervous System

, Volume 34, Issue 5, pp 861–870 | Cite as

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

  • Khalid AbdallaEmail author
  • Shaker Abdullah
  • Abeer Almehdar
  • Naglla Elimam
  • Mohammed Burhan Abrar
  • Wasil Jastaniah
Original Paper



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.


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).


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.


LGG Children Outcome Surgery Developing countries 


Compliance with ethical standards

Conflict of interest

The authors have no conflicts of interest to disclose.


  1. 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, LyonGoogle Scholar
  2. 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. CrossRefGoogle Scholar
  3. 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. CrossRefPubMedGoogle Scholar
  4. 4.
    Schmandt SM, Packer RJ (2000) Treatment of low-grade pediatric gliomas. Curr Opin Oncol 12(3):194–198. CrossRefPubMedGoogle Scholar
  5. 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. CrossRefPubMedGoogle Scholar
  6. 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):347–353Google Scholar
  7. 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):1265–1284CrossRefPubMedPubMedCentralGoogle Scholar
  8. 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):1257–1268Google Scholar
  9. 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 30:1358–1363CrossRefPubMedGoogle Scholar
  10. 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 34:3537–3543CrossRefPubMedGoogle Scholar
  11. 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 10:452Google Scholar
  12. 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. CrossRefPubMedGoogle Scholar
  13. 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):835–840Google Scholar
  14. 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.
  15. 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. CrossRefPubMedGoogle Scholar
  16. 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. CrossRefPubMedGoogle Scholar
  17. 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. CrossRefPubMedGoogle Scholar
  18. 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. CrossRefGoogle Scholar
  19. 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:
  20. 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):104–108CrossRefPubMedGoogle Scholar
  21. 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):1173–1179CrossRefPubMedPubMedCentralGoogle Scholar
  22. 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):354–362PubMedGoogle Scholar
  23. 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. CrossRefGoogle Scholar
  24. 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):2131–2140Google Scholar
  25. 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. CrossRefPubMedGoogle Scholar
  26. 26.
    Baumert BG, Stupp R (2010) Is there a place for radiotherapy in low-grade gliomas? Adv Tech Stand Neurosurg 35:159–182CrossRefPubMedGoogle Scholar
  27. 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. CrossRefPubMedGoogle Scholar
  28. 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. CrossRefPubMedGoogle Scholar
  29. 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. CrossRefPubMedGoogle Scholar
  30. 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 57:84–88CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Khalid Abdalla
    • 1
    Email author
  • Shaker Abdullah
    • 1
  • Abeer Almehdar
    • 1
  • Naglla Elimam
    • 1
  • Mohammed Burhan Abrar
    • 1
  • Wasil Jastaniah
    • 1
    • 2
  1. 1.Princess Noorah Oncology CenterKing Saud Bin Abdulaziz University and King Abdulaziz Medical CityJeddahSaudi Arabia
  2. 2.Department of Pediatrics, College of MedicineUmm AlQura UniversityMakkahSaudi Arabia

Personalised recommendations