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Atypical teratoid rhabdoid tumours (ATRTs)-a 21-year institutional experience.
Silva, A H D; Habermann, S; Craven, C L; Bhagawati, D; O'Hare, P; Jorgensen, M; Dahl, C; Mankad, K; Thompson, D N P; Hargrave, D; Jeelani, N Ul Owase; Aquilina, K.
Afiliación
  • Silva AHD; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Habermann S; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Craven CL; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Bhagawati D; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • O'Hare P; Department of Paediatric Oncology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Jorgensen M; Department of Paediatric Oncology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Dahl C; Department of Paediatric Oncology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Mankad K; Department of Paediatric Neuroradiology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Thompson DNP; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Hargrave D; Department of Paediatric Oncology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Jeelani NUO; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK.
  • Aquilina K; Department of Paediatric Neurosurgery, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, WC1N 3JH, UK. Kristian.Aquilina@gosh.nhs.uk.
Childs Nerv Syst ; 39(6): 1509-1518, 2023 06.
Article en En | MEDLINE | ID: mdl-36790496
PURPOSE: 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. METHODS: Retrospective cohort review of patients with histological diagnosis of ATRT treated over 21 years (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. RESULTS: A total of 45 children (mean age 2 years) underwent 64 operations. 25 patients were <1 year 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 <1 year vs >1 year of age (p=0.84). CONCLUSION: We find that presence of metastasis was an important factor in poor survival in patients with ATRT. GTR, where possible, may confer significant survival benefit in ATRT. Children aged <1 year appear to have performed as well as those >1 year and therefore should still be considered for radical surgery.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Teratoma / Neoplasias del Sistema Nervioso Central / Tumor Rabdoide Tipo de estudio: Observational_studies Límite: Child / Child, preschool / Humans Idioma: En Revista: Childs Nerv Syst Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2023 Tipo del documento: Article Pais de publicación: Alemania
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Teratoma / Neoplasias del Sistema Nervioso Central / Tumor Rabdoide Tipo de estudio: Observational_studies Límite: Child / Child, preschool / Humans Idioma: En Revista: Childs Nerv Syst Asunto de la revista: NEUROLOGIA / PEDIATRIA Año: 2023 Tipo del documento: Article Pais de publicación: Alemania