Pediatric low-grade glioma in Africa: a baseline study before the implementation of Global Initiative for Childhood Cancer strategies

Introduction Pediatric low-grade glioma (LGG) is a World Health Organization (WHO) Global Initiative for Childhood Cancer (GICC) index tumor constituting up to a third of pediatric central nervous system (CNS) tumors. The baseline characteristics, survival, and management resources for pediatric LGG in Africa are unknown. We aimed to evaluate the pediatric neuro-oncology multidisciplinary team resources, epidemiology, and survival outcomes of pediatric LGG in Africa to document baseline information prior to GICC implementation. Methods The study consisted of two parts: a survey completed by African pediatric oncology units (POU) to evaluate the local resources and a retrospective evaluation of data to determine the 5-year overall survival (OS) for patients under 18 years diagnosed with LGG between 2008 and 2018. Data were described in frequencies and percentages. Survival was expressed with Kaplan–Meier curves. Results Five-hundred and eighty-eight patients were included from fifteen POUs in six countries: South Africa (45.9%), Egypt (30.8%), Morocco (12.6%), Mali (4.4%), Tunisia (3.6%) and Uganda (2.7%). The median age was 4.4 years (interquartile range 2.4–7.3 years). The most common primary tumor site was the brainstem ( n = 125, 21.3%), the most common histology was pilocytic astrocytoma ( n = 270, 47.5%), the majority of tumors ( n = 292, 93%) were localized, and 40 (6.8%) patients had neurofibromatosis. Complete resection was obtained in 99 (16.8%) cases, incomplete resection in 179 (30.4%), and no surgery or biopsy only was performed in 310 (52.7%) cases. One hundred and forty-seven (25.3%) of the 580 patients with a documented radiotherapy status, were irradiated, and 320 (54.4%) received chemotherapy. Only 259 (15.3%) patients received chemotherapy of which the most common chemotherapy regimen was vincristine-carboplatin ( n = 220, 84.9%). The 5-year OS was 90.5% ± 1.6%. The 5-year OS in Tunisia was 95.1% ± 1.1%, 92.4% ± 2.1% in Egypt, 89.0% ± 3.2% in South Africa, 70.7% ± 6.7% in Morocco and 66.7% ± 15.7% in Uganda ( p < 0.001). Four of the 41 (9.8%) responding countries reported having pediatric neuro-oncology subspecialists, and four (9.8%) had national pediatric LGG protocols. In Africa there is one radiotherapy center per 2,235,125 children and one neurosurgeon per 304,685 children, with ~70% of these resources accessible in four countries. Discussion Due to several resource challenges and developing treatment centers, only fifteen pediatric oncology units from six countries participated. We documented a baseline 5-year OS of 94.9% for LGG in African children. To obtain an accurate estimation of pediatric LGG survival in Africa, increasing participation from a wider range of countries, especially poorly resourced settings, is necessary.