title: Neurocognitive evaluation of long term survivors of atypical teratoid rhabdoid tumors (ATRT): The Canadian registry experience.
abstract: BACKGROUND
  Because atypical teratoid rhabdoid tumor(ATRT) is a rare disease of infancy carrying a grim prognosis, focus on long-term outcome, especially neurocognitive remained very limited. With new era of multimodality therapy, an increasing proportion of patients are now long-term survivors.
  
  
  METHODS
  Retrospective review of neuropsychological (NP) status of survivors from the Canadian ATRT registry.
  
  
  RESULTS
  Among 77 patients diagnosed between 1995-2012, 16(22%) were survivors. Formal NP assessments were available in eight patients. Partial information on academic achievement was available on three additional patients. There were four girls and seven boys diagnosed at a median age of 27.5 months. Seven patients underwent complete resection and three had metastatic disease. All but one received sequential high dose chemotherapy. Five patients received intrathecal chemotherapy. Three patients underwent radiation. Median age at time of formal NP assessment was 7.6 years (3.9-9.8). Full Scale Intellectual Quotient (FSIQ) ranged from less than 50-119 (mean 78). Simple expressive and receptive language appeared relatively preserved. Three recently diagnosed patients (median time assessment from diagnosis 2.6 years (1.6-4.7)) had average to high average FSIQ, academic and visual spatial skills, visual, and verbal memory. Five other patients diagnosed earlier and tested at a median time of 4.9 years (3.3-8.3) post-diagnosis had a FSIQ ranging from <50 to 71. Approximately 50% of their scores were in the impaired range.
  
  
  CONCLUSIONS
  Overall this cohort appears significantly impaired at school age despite the absence of systematic radiotherapy. Larger series focusing on neurocognitive outcome are needed in the current context where treatment strategies include adjuvant radiation.
authors: Division of Hematology Oncology Bone Marrow Transplantation, Alberta Children's Hospital, Calgary, Alberta, Canada.
authors: Lucie
authors: L
authors: Lafay-Cousin
authors: Division of Hematology Oncology Bone Marrow Transplantation, Alberta Children's Hospital, Calgary, Alberta, Canada.
authors: Taryn
authors: T
authors: Fay-McClymont
authors: Division of Hematology/Oncology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors: Donna
authors: D
authors: Johnston
authors: Children and Women Hospital of British Columbia, Vancouver, British Columbia, Canada.
authors: Chris
authors: C
authors: Fryer
authors: Mc Master University Hospital, Hamilton, Ontario, Canada.
authors: Katrin
authors: K
authors: Scheinemann
authors: Children's Hospital of Montreal, Montreal, Quebec, Canada.
authors: Adam
authors: A
authors: Fleming
authors: Children and Women Hospital of British Columbia, Vancouver, British Columbia, Canada.
authors: Juliette
authors: J
authors: Hukin
authors: Department of Psychology, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Laura
authors: L
authors: Janzen
authors: Department of Psychology, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Sharon
authors: S
authors: Guger
authors: Division of Hematology Oncology Bone Marrow Transplantation, Alberta Children's Hospital, Calgary, Alberta, Canada.
authors: Douglas
authors: D
authors: Strother
authors: Department of Psychology, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Donald
authors: D
authors: Mabbott
authors: Pediatric Brain Tumor Program, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Annie
authors: A
authors: Huang
authors: Pediatric Brain Tumor Program, Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Eric
authors: E
authors: Bouffet
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000523:psychology; D002170:Canada; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology / Q000523:psychology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009483:Neuropsychological Tests; D011379:Prognosis; D012042:Registries / Q000706:statistics & numerical data*; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000150:complications / Q000523:psychology*; D015996:Survival Rate; D017741:Survivors / Q000523:psychology*; D013724:Teratoma / Q000150:complications / Q000523:psychology*
keywords: ATRT; neurocognitive evaluation; survivor