Abstract
Primary CNS tumors are the leading cause of cancer-related death in pediatrics. It is essential to understand treatment trends to interpret national survival data. In Canada, children with CNS tumors are treated at one of 16 tertiary care centers. We surveyed pediatric neuro-oncologists to create a national standard of practice to be used in the absence of a clinical trial for seven of the most prevalent brain tumors in children. This allowed description of practice across the country, along with a consensus. This had a multitude of benefits, including understanding practice patterns, allowing for a basis to compare in future research and informing Health Canada of the current management of patients. This also allows all children in Canada to receive equivalent care, regardless of location.
Highlights
Primary central nervous system (CNS) tumors are the 2nd most common cancer and the leading cause of cancer-related death in children [1, 2]
A survey was sent to all Canadian pediatric oncology institutions to collect data on which protocol would be used in the absence of a clinical trial for common pediatric CNS tumors including pediatric low grade glioma (pLGG) (1st and 2nd line therapy), pediatric high grade glioma, childhood and infant MB, EPN, Atypical Teratoid Rhabdoid Tumors (ATRT), craniopharyngioma and CNS germ cell tumor (GCT, germinoma and non-germinomatous germ cell tumor (NGGCT))
Results from ACNS1221 were recently published studying outcomes using the HIT SKK 2000 regimen but omitting intrathecal methotrexate. It was a negative trial, patients with medulloblastoma with extensive nodularity (MBEN) histologies (n=7) had 100% progression-free survival (PFS), and this regimen could be considered for a child ≤ 1 year [54]
Summary
Primary central nervous system (CNS) tumors are the 2nd most common cancer and the leading cause of cancer-related death in children [1, 2]. Atypical Teratoid Rhabdoid Tumors (ATRT) are highly malignant embryonal brain tumors arising predominantly in very young children (median age of diagnosis 2 years). Historical clinical trials by the Pediatric Oncology Group (POG) 9233/4 and Children’s Cancer Group (CCG) 9921 [79, 80], treated very young children diagnosed with various embryonal brain tumors including ATRTs with multi-agent chemotherapy, resulting in a dismal EFS of 6.4%. Craniopharyngiomas (CPs) are mixed solid-cystic tumors that account for approximately 5%–10% of brain tumors in children [94, 95] and are one of the most frequently diagnosed hypothalamo-pituitary lesions in this age group [95] They are defined histologically as benign epithelial tumors of the sellar region and postulated to arise from embryonic remnants of Rathke’s pouch during development of the fetal adenohypophysis [96]. Germinomas can produce small amounts of the b subunit of human chorionic gonadotropin (bHCG) and a level less than 100 IU/L in serum and CSF is accepted in North America [113]
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