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PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA
OBJECTIVE: To compare the experience with pinealoblastoma (PB) of the neuro-oncology services of the University of Cape Town to the WHO 2021 classification. METHODS: A retrospective analysis was performed on folders of patients diagnosed at Red Cross War Memorial Children’s Hospital and Groote Schuu...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10616585/ http://dx.doi.org/10.1093/noajnl/vdad121.043 |
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author | Davidson, Alan Ohonba, Efosa Parkes, Jeannette Pillay, Komala Figaji, Anthony |
author_facet | Davidson, Alan Ohonba, Efosa Parkes, Jeannette Pillay, Komala Figaji, Anthony |
author_sort | Davidson, Alan |
collection | PubMed |
description | OBJECTIVE: To compare the experience with pinealoblastoma (PB) of the neuro-oncology services of the University of Cape Town to the WHO 2021 classification. METHODS: A retrospective analysis was performed on folders of patients diagnosed at Red Cross War Memorial Children’s Hospital and Groote Schuur Hospital from 2000-2022. Routine molecular testing is not available nor is testing for DICER mutations. Patients with PB associated with retinoblastoma (RB) were assumed to be Rb1-altered and were treated with ophthalmic local control and chemotherapy, and those without associated RB (assumed to be non-Rb1-altered) were mostly treated with surgery, chemotherapy and craniospinal radiotherapy. Convenience sampling was performed by age with respect to disease characteristics and outcome to try and disaggregate miRNA-processing-altered-1 and -2 from MYC/FOXR2-altered PB. RESULTS: Twenty-five patients were identified, 6 with Rb1-altered PB, aged 0.33-1.88 years (median 0.79) and 19 with non-Rb1-altered PB, aged 0.17-12.99 years (median 6.23). The whole cohort was balanced in terms of gender but there were slightly more girls (11) than boys (8) among the non-Rb1-altered PBs. One of these children was a 5-year-old boy with DICER syndrome who was identified through family screening. One Rb1-altered PB had metastatic disease outside the pineal gland. Among the non-Rb1-altered PBs 47% had metastases, 0% of those under 3 years, 40% of those aged 3-9 years and 80% of those older than 9 years. Estimated 5-year OS for the whole group was 49%, 50% for Rb1-altered PB and 49% for non-Rb1-altered PBs. OS by age among non-Rb1-altered PBs was 0% for 0-3 years, 90% for 3-9 years and 20% for those older than 9 years. CONCLUSION: DICER testing and molecular typing would be very useful in helping to stratify our non-Rb1-altered PBs, both for prognostication and to allow consideration of high-risk strategies. Multi-disciplinary treatment of PB in LMIC is essential. |
format | Online Article Text |
id | pubmed-10616585 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-106165852023-11-01 PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA Davidson, Alan Ohonba, Efosa Parkes, Jeannette Pillay, Komala Figaji, Anthony Neurooncol Adv Final Category: Paediatric Oncology OBJECTIVE: To compare the experience with pinealoblastoma (PB) of the neuro-oncology services of the University of Cape Town to the WHO 2021 classification. METHODS: A retrospective analysis was performed on folders of patients diagnosed at Red Cross War Memorial Children’s Hospital and Groote Schuur Hospital from 2000-2022. Routine molecular testing is not available nor is testing for DICER mutations. Patients with PB associated with retinoblastoma (RB) were assumed to be Rb1-altered and were treated with ophthalmic local control and chemotherapy, and those without associated RB (assumed to be non-Rb1-altered) were mostly treated with surgery, chemotherapy and craniospinal radiotherapy. Convenience sampling was performed by age with respect to disease characteristics and outcome to try and disaggregate miRNA-processing-altered-1 and -2 from MYC/FOXR2-altered PB. RESULTS: Twenty-five patients were identified, 6 with Rb1-altered PB, aged 0.33-1.88 years (median 0.79) and 19 with non-Rb1-altered PB, aged 0.17-12.99 years (median 6.23). The whole cohort was balanced in terms of gender but there were slightly more girls (11) than boys (8) among the non-Rb1-altered PBs. One of these children was a 5-year-old boy with DICER syndrome who was identified through family screening. One Rb1-altered PB had metastatic disease outside the pineal gland. Among the non-Rb1-altered PBs 47% had metastases, 0% of those under 3 years, 40% of those aged 3-9 years and 80% of those older than 9 years. Estimated 5-year OS for the whole group was 49%, 50% for Rb1-altered PB and 49% for non-Rb1-altered PBs. OS by age among non-Rb1-altered PBs was 0% for 0-3 years, 90% for 3-9 years and 20% for those older than 9 years. CONCLUSION: DICER testing and molecular typing would be very useful in helping to stratify our non-Rb1-altered PBs, both for prognostication and to allow consideration of high-risk strategies. Multi-disciplinary treatment of PB in LMIC is essential. Oxford University Press 2023-10-31 /pmc/articles/PMC10616585/ http://dx.doi.org/10.1093/noajnl/vdad121.043 Text en © The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Final Category: Paediatric Oncology Davidson, Alan Ohonba, Efosa Parkes, Jeannette Pillay, Komala Figaji, Anthony PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA |
title | PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA |
title_full | PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA |
title_fullStr | PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA |
title_full_unstemmed | PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA |
title_short | PAEDIATRIC-11 EPIDEMIOLOGY OF PINEALOBLASTOMA IN CAPE TOWN, SOUTH AFRICA |
title_sort | paediatric-11 epidemiology of pinealoblastoma in cape town, south africa |
topic | Final Category: Paediatric Oncology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10616585/ http://dx.doi.org/10.1093/noajnl/vdad121.043 |
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