title: 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.
authors.affiliation: Pediatric Hematology Oncology and Bone Marrow Transplantation, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.
authors.forename: Mary
authors.initials: M
authors.lastname: Fossey
authors.affiliation: Pediatric Hematology Oncology and Bone Marrow Transplantation, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.
authors.forename: Haocheng
authors.initials: H
authors.lastname: Li
authors.affiliation: Hematology Oncology, IWK Health Centre, 5850 University Avenue, P O Box 9700, Halifax, NS, B3K 6R8, Canada.
authors.forename: Samina
authors.initials: S
authors.lastname: Afzal
authors.affiliation: Hematology Oncology, Centre Hospitalier Universitaire Sainte-Justine, Pavillon Charles Bruneau, A.12.39, 3175 chemin Cote Sainte-Catherine, Montreal, QC, H1T 3C5, Canada.
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Carret
authors.affiliation: Pediatric Oncology, Stollery Children's Hospital, Aberhart Centre One, 11402 University Ave, Edmonton, AB, T6G 1C9, Canada.
authors.forename: David D
authors.initials: DD
authors.lastname: Eisenstat
authors.affiliation: Department of Pediatrics, McMaster Children's Hospital, 1280 Main Street West, Hamilton, ON, L8S 4K1, Canada.
authors.forename: Adam
authors.initials: A
authors.lastname: Fleming
authors.affiliation: Neurology-Oncology, British Columbia Children's Hospital, 4480 Oak Street, Room B315, Vancouver, BC, V6H 3V4, Canada.
authors.forename: Juliette
authors.initials: J
authors.lastname: Hukin
authors.affiliation: Pediatric Laboratory Medicine, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
authors.forename: Cynthia
authors.initials: C
authors.lastname: Hawkins
authors.affiliation: Pediatrics, The Montreal Children's Hospital of the MUHC, 4060 Ste Catherine West, Montreal, QC, H3Z 2Z3, Canada.
authors.forename: Nada
authors.initials: N
authors.lastname: Jabado
authors.affiliation: Hematology Oncology, Children's Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, ON, K1H 8L1, Canada.
authors.forename: Donna
authors.initials: D
authors.lastname: Johnston
authors.affiliation: Pediatric Oncology, Saskatoon Cancer Centre, #20 Campus Drive, Saskatoon, SK, S7N 4H4, Canada.
authors.forename: Tania
authors.initials: T
authors.lastname: Brown
authors.affiliation: Pediatric Hematology Oncology, Centre Hospitalier de l'Université Laval (CHUL), 2705, boulevard Laurier, Quebec, G1V 4G2, Canada.
authors.forename: Valerie
authors.initials: V
authors.lastname: Larouche
authors.affiliation: Department of Pediatrics, McMaster Children's Hospital, 1280 Main Street West, Hamilton, ON, L8S 4K1, Canada.
authors.forename: Katrin
authors.initials: K
authors.lastname: Scheinemann
authors.affiliation: Pediatric Hematology Oncology and Bone Marrow Transplantation, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.
authors.forename: Douglas
authors.initials: D
authors.lastname: Strother
authors.affiliation: Pediatric Oncology, Stollery Children's Hospital, Aberhart Centre One, 11402 University Ave, Edmonton, AB, T6G 1C9, Canada.
authors.forename: Beverly
authors.initials: B
authors.lastname: Wilson
authors.affiliation: Pediatrics, London Children's Hospital, 800 Commissioner's Road East, London, ON, N6C 2V5, Canada.
authors.forename: Shayna
authors.initials: S
authors.lastname: Zelcer
authors.affiliation: Pediatric Brain Tumor Program, Hematology/Oncology, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1XB, Canada.
authors.forename: Annie
authors.initials: A
authors.lastname: Huang
authors.affiliation: Pediatric Brain Tumor Program, Hematology/Oncology, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1XB, Canada.
authors.forename: Eric
authors.initials: E
authors.lastname: Bouffet
authors.affiliation: Pediatric Hematology Oncology and Bone Marrow Transplantation, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada. lucie.lafay-cousin@ahs.ca.
authors.forename: Lucie
authors.initials: L
authors.lastname: Lafay-Cousin
mesh_terms: D002170:Canada; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D010166:Palliative Care / Q000706:statistics & numerical data; D018714:Radiotherapy, Adjuvant; D012042:Registries; D018335:Rhabdoid Tumor / Q000453:epidemiology* / Q000532:radiotherapy / Q000601:surgery; D013724:Teratoma / Q000453:epidemiology* / Q000532:radiotherapy / Q000601:surgery; D016896:Treatment Outcome
keywords: ATRT; Germline mutation; High dose chemotherapy; Infant