نبذة مختصرة : Background: Atypical teratoid/rhabdoid tumours is a relatively rare malignant neoplasm in the paediatric population. It is characterized by the presence of rhabdoid cells in combination with loss of either the INI1 or BRG1 protein from the tumour cells. Aim: To systematically review and analyse patient and tumour characteristics, prognosis, and impact of treatment on survival in paediatric patients with Atypical Teratoid/Rhabdoid Tumour (AT/RT) confirmed by alterations in INI1 or BRG1. This is the first systematic review to only include cases of AT/RT confirmed with either INI1 or BRG1 alterations. Methods: A systematic review was conducted using MEDLINE using the terms “atypical teratoid rhabdoid tumour” AND “paediatric”. Cases were included if the pathology was confirmed by loss of INI1 or BRG1. The dataset was analysed using descriptive statistics, log-rank test, and Kaplan-Meier survival analysis via SPSS. Results: A total of 38 articles were included in this study. The average age at diagnosis was 3 years. The most common locations reported is the supratentorial region and cerebral hemispheres. 93 patients were reported to show evidence of dissemination. The average overall survival was 29 months. There was a significant difference in survival between the tumour location groups, particularly worst outcomes for patients with spinal AT/RT (p = <0.001), but not statistically significant differences in adjuvant therapy groups (p = 0.581) and the extent of surgical resection groups (p= 0.262). Conclusion: Atypical teratoid rhabdoid tumour of the central nervous system in paediatric populations is a rare neoplasm associated with a poor prognosis in most patients. The treatment and clinical course are highly variable, and it remains unclear which factors impact prognosis.
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