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. 2023 Jun;39(6):1509-1518.
doi: 10.1007/s00381-023-05828-0. Epub 2023 Feb 15.

Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience

A H D Silva #  1 , S Habermann #  1 , C L Craven #  1 , D Bhagawati  1 , P O'Hare  2 , M Jorgensen  2 , C Dahl  2 , K Mankad  3 , D N P Thompson  1 , D Hargrave  2 , N Ul Owase Jeelani  1 , K Aquilina  4
Affiliations

Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience

A H D Silva et al. Childs Nerv Syst. 2023 Jun.

Abstract

Purpose: Atypical 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.

Methods: Retrospective 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.

Results: A 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).

Conclusion: We 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.

Keywords: ATRT; Posterior fossa tumour; Survival analysis.

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References

    1. Frühwald MC, Biegel JA, Bourdeaut F, Roberts CW, Chi SN (2016) Atypical teratoid/rhabdoid tumors-current concepts, advances in biology, and potential future therapies. Neuro Oncol 18(6):764–78 - DOI - PubMed - PMC
    1. Woehrer A, Slavc I, Waldhoer T, Heinzl H, Zielonke N, Czech T, Benesch M, Hainfellner JA, Haberler C (2010) Incidence of atypical teratoid/rhabdoid tumors in children: a population-based study by the Austrian Brain Tumor Registry, 1996–2006 Austrian Brain Tumor Registry. Cancer 116(24):5725–32 - DOI - PubMed
    1. Rorke LB, Packer RJ, Biegel JA (1996) Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 85:56–65 - DOI - PubMed
    1. Biegel JA (2006) Molecular genetics of atypical teratoid/rhabdoid tumor. Neurosurg Focus 20(1):E11 - DOI - PubMed
    1. Richardson EA, Ho B, Huang A (2018) Atypical Teratoid Rhabdoid Tumour : From Tumours to Therapies. J Korean Neurosurg Soc 61(3):302–311. https://doi.org/10.3340/jkns.2018.0061 - DOI - PubMed - PMC

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