Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience.

Abstract

Atypical teratoid/rhabdoid tumours (ATRTs) are malignant embryonal tumours of childhood that affect the central nervous system (CNS). We aim to determine which factors, including patient age, extent of resection (EOR), presence of distal metastasis and use of adjuvant therapies, affect overall survival in children with atypical teratoid/rhabdoid tumours (ATRTs) treated at this single centre. Retrospective cohort review of patients with histological diagnosis of ATRT treated over 21years (1999-2020) was conducted. Data on demographics, tumour location, presence of metastasis, use of adjuvant therapy, extent of resection (EOR), complications, neurological outcome post-surgery, and overall survival were collected. Kaplan-Meier survival analysis was performed. A total of 45 children (mean age 2 years) underwent 64 operations. 25 patients were <1year of age. Gross-total resection (GTR) pre-adjuvant therapy was achieved in 15, near-total resection (NTR) in 15, subtotal resection (STR) in 9, and biopsy in 6 children. Most children had good neurological outcomes post-operatively (28/45 with GOS 5). Fourteen patients survived longer than 4 years. Survival analysis showed a significant difference in median survival in favour of GTR and localised disease. There was no significant difference in median survival between patients <1year vs >1year of age (p=0.84). We find that presence of metastasis was an important factor in poor survival inpatients with ATRT. GTR, where possible, may confer significant survival benefit in ATRT. Children aged <1year appear to have performed as well as those >1year and therefore should stillbe considered for radical surgery.