ArticleChild's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2023
Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience.
Article in Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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4 citing papers in PubMed, 6 citations in OpenAlex.
- Histogenesis of Atypical Teratoid Rhabdoid Tumors: Anatomical and Embryological Perspectives.Cancers · 2025Article
- Characterizing the Natural History of Pediatric Brain Tumors Presenting with Metastasis.Cancers · 2025Article
- Pediatric Posterior Fossa ATRT: A Case Report, New Treatment Strategies and Perspectives.Brain sciences · 2023Article
- Current Molecular and Clinical Landscape of ATRT - The Link to Future Therapies.Cancer management and research · 2023Review
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12 authors at 1 institution in 1 country.
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Abstract
purposeAtypical teratoid/rhabdoid tumours (ATRTs) are malignant embryonal tumours of childhood that affect the central nervous system (CNS). We aim to determine which factors, including patient age, extent of resection (EOR), presence of distal metastasis and use of adjuvant therapies, affect overall survival in children with atypical teratoid/rhabdoid tumours (ATRTs) treated at this single centre.
methodsRetrospective cohort review of patients with histological diagnosis of ATRT treated over 21 years (1999-2020) was conducted. Data on demographics, tumour location, presence of metastasis, use of adjuvant therapy, extent of resection (EOR), complications, neurological outcome post-surgery, and overall survival were collected. Kaplan-Meier survival analysis was performed.
resultsA total of 45 children (mean age 2 years) underwent 64 operations. 25 patients were <1 year of age. Gross-total resection (GTR) pre-adjuvant therapy was achieved in 15, near-total resection (NTR) in 15, subtotal resection (STR) in 9, and biopsy in 6 children. Most children had good neurological outcomes post-operatively (28/45 with GOS 5). Fourteen patients survived longer than 4 years. Survival analysis showed a significant difference in median survival in favour of GTR and localised disease. There was no significant difference in median survival between patients <1 year vs >1 year of age (p=0.84).
conclusionWe find that presence of metastasis was an important factor in poor survival in patients with ATRT. GTR, where possible, may confer significant survival benefit in ATRT. Children aged <1 year appear to have performed as well as those >1 year and therefore should still be considered for radical surgery.
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