Neurocognitive evaluation of long term survivors of atypical XXXX (ATRT): The Canadian registry experience.

@entity1 @entity227 @entity5 @entity5606 @entity3
BACKGROUND: Because atypical @entity5606 (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 @entity1 are now long-term survivors. PROCEDURE: Retrospective review of neuropsychological (NP) status of survivors from the Canadian ATRT registry. RESULTS: Among 77 @entity1 diagnosed between 1995-2012, 16(22%) were survivors. Formal NP assessments were available in eight @entity1 . Partial information on academic achievement was available on three additional @entity1 . There were four @entity1 and seven @entity1 diagnosed at a median age of 27.5 months. Seven @entity1 underwent complete resection and three had @entity3 . All but one received sequential high dose chemotherapy. Five @entity1 received intrathecal chemotherapy. Three @entity1 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 @entity1 (median time assessment from diagnosis 2.6 years (1.6-4.7)) had average to high average FSIQ, @entity227 . Five other @entity1 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. CONCLUSION: 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. Pediatr @entity5    2015 Wiley Periodicals, Inc.
