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

摘要: Background Because atypical teratoid rhabdoid tumor(ATRT) is a rare disease of infancy carrying grim prognosis, focus on long-term outcome, especially neurocognitive remained very limited. With new era multimodality therapy, an increasing proportion patients are now survivors. Procedure Retrospective review neuropsychological (NP) status survivors from the Canadian ATRT registry. Results Among 77 diagnosed between 1995–2012, 16(22%) were survivors. Formal NP assessments available in eight patients. Partial information academic achievement was three additional There four girls and seven boys at median age 27.5 months. Seven underwent complete resection had metastatic disease. All but one received sequential high dose chemotherapy. Five intrathecal Three radiation. Median time formal assessment 7.6 years (3.9–9.8). Full Scale Intellectual Quotient (FSIQ) ranged less than 50–119 (mean 78). Simple expressive receptive language appeared relatively preserved. recently (median diagnosis 2.6 (1.6–4.7)) average to FSIQ, visual spatial skills, visual, verbal memory. other earlier tested 4.9 (3.3–8.3) post-diagnosis FSIQ ranging <50 71. Approximately 50% their scores impaired range. Conclusion Overall this cohort appears significantly school despite absence systematic radiotherapy. Larger series focusing outcome needed current context where treatment strategies include adjuvant Pediatr Blood Cancer 2015;62:1265–1269. © 2015 Wiley Periodicals, Inc.