Atypical teratoid rhabdoid tumor in the first year of life: the Canadian ATRT registry experience and review of the literature

Abstract

While 2/3 of patients with ATRT are less than 3 years at diagnosis, the literature suggests younger children present with more aggressive disease and poorer outcome. However, little data exist on characteristics and outcome of patients diagnosed with ATRT in the first year of life. In particular, it is unclear whether they access similar treatments as do older children. We compared the cohort of patients ≤12 months from the Canadian ATRT registry to all cases extracted from the literature reported between 1996 and 2014 to describe their clinical and treatment characteristics, and potential prognostic factors. Twenty-six (33.7%) patients from the Canadian registry were ≤12 months at diagnosis as were 120 cases identified in the literature. Post-operatively, 46% of the registry’s patients underwent palliation as opposed to 10.8% in the literature cohort. Palliative patients were significantly younger than those who received active therapy (3.3 vs. 6.6 months). While the use of high-dose chemotherapy (HDC) was relatively similar in both cohorts (42.9 and 35.5% respectively), radiotherapy (RT) use was significantly lower in the Canadian cohort (14.3 vs 44.9%). Children ≤6 months, who received active therapy, had a worst outcome than older ones. Gross total resection, HDC and adjuvant RT were associated with better outcomes. Eighty percent of the tested patients had evidence of germline mutation of INI1. While 1/3 of ATRT occurs within the first year of life, a large proportion only received palliative therapy. Even when actively treated, children ≤6 months fare worse. Some selected patients benefit from HDC.

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Funding

This work was supported by a summer research studentship for M. F., from the Maternal, Newborn, Child and Youth Strategic Clinical Network, Alberta Health Services, and the Alberta Children’s Hospital Research Institute. The establishment of the Canadian ATRT registry was supported by the C17 and funded [or partially funded] by Kids With Cancer Society, Childhood Cancer Canada Foundation, and Coast to Coast Against Cancer Foundation.

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Correspondence to Lucie Lafay-Cousin.

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None of the authors have conflicts of interest to declare.

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All participating Canadian centers received research ethics board approval to contribute to the Canadian ATRT registry.

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Fossey, M., Li, H., Afzal, S. et al. Atypical teratoid rhabdoid tumor in the first year of life: the Canadian ATRT registry experience and review of the literature. J Neurooncol 132, 155–162 (2017). https://doi.org/10.1007/s11060-016-2353-0

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Keywords

  • ATRT
  • Infant
  • High dose chemotherapy
  • Germline mutation