ORIGINAL PAPER Challenges in the management of childhood low-grade glioma in a developing country Khalid Abdalla 1 & Shaker Abdullah 1 & Abeer Almehdar 1 & Naglla Elimam 1 & Mohammed Burhan Abrar 1 & Wasil Jastaniah 1,2 Received: 20 November 2017 /Accepted: 16 January 2018 # Springer-Verlag GmbH Germany, part of Springer Nature 2018 Abstract Background Treatment modality impacts outcome of childhood low-grade glioma (LGG). Optimizing management in develop- ing 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 corre- sponding 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. Keywords LGG . Children . Outcome . Surgery . Developing countries Introduction Low-grade gliomas (LGGs) comprise 30–50% of brain tu- mors in children, occurring anywhere within the central ner- vous system (CNS). The commonest are astrocytic tumors with pilocytic astrocytoma being the most common [1, 2]. Cerebellar astrocytoma is the most prevalent site accounting for 15–25% of all CNS tumors in children [1, 2]. Treatment strategies for childhood LGG have changed con- siderably over the past few decades. Although observation is an option, particularly for patients with neurofibromatosis type 1 (NF1) and/or non-progressive tumors, surgical resection remains the gold standard and is considered curative, especially when complete resection is achieved [3–5]. The ability to achieve com- plete resection is largely dependent on tumor location, complete resection rates therefore varying between studies [6–8]. Chemotherapy is used both as salvage therapy for progres- sive disease and as first-line therapy, particularly given the efficacy and safety of low-toxicity regimens [9–11]. Radiotherapy is reserved for recurrent/progressive tumors due to the significant late sequalae [12]. Hence, radiation/ * Khalid Abdalla ps.remed@gmail.com; Abdallaka@ngha.med.sa 1 Princess Noorah Oncology Center, King Saud Bin Abdulaziz University and King Abdulaziz Medical City, P.O.BOX 9515, Jeddah 21423, Saudi Arabia 2 Department of Pediatrics, College of Medicine, Umm AlQura University, Makkah, Saudi Arabia Child's Nervous System https://doi.org/10.1007/s00381-018-3729-6