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Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience
BACKGROUND: Adult medulloblastoma (MB) is rare, and management guidelines are largely based on pediatric clinical trials and retrospective series. Limited data exist with respect to clinical characteristics, prognostic factors, and outcomes based on first-line treatments. METHODS: Two hundred adults...
Autores principales: | , , , , , , , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8350154/ https://www.ncbi.nlm.nih.gov/pubmed/34377987 http://dx.doi.org/10.1093/noajnl/vdab079 |
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author | Majd, Nazanin K Mastall, Maximilan Lin, Heather Dibaj, Seyede Shiva Hess, Kenneth R Yuan, Ying Garcia, Manuela Martin-Bejarano Fuller, Gregory N Alfaro, Kristin D Gule-Monroe, Maria K Huse, Jason T Khatua, Soumen Rao, Ganesh Sandberg, David I Wefel, Jeffrey S Yeboa, Debra N Paulino, Arnold C McGovern, Susan L Zaky, Wafik Mahajan, Anita Suki, Dima Weathers, Shiao-Pei Harriso, Rebecca A De Groo, John F Puduvalli, Vinay K Penas-Prado, Marta |
author_facet | Majd, Nazanin K Mastall, Maximilan Lin, Heather Dibaj, Seyede Shiva Hess, Kenneth R Yuan, Ying Garcia, Manuela Martin-Bejarano Fuller, Gregory N Alfaro, Kristin D Gule-Monroe, Maria K Huse, Jason T Khatua, Soumen Rao, Ganesh Sandberg, David I Wefel, Jeffrey S Yeboa, Debra N Paulino, Arnold C McGovern, Susan L Zaky, Wafik Mahajan, Anita Suki, Dima Weathers, Shiao-Pei Harriso, Rebecca A De Groo, John F Puduvalli, Vinay K Penas-Prado, Marta |
author_sort | Majd, Nazanin K |
collection | PubMed |
description | BACKGROUND: Adult medulloblastoma (MB) is rare, and management guidelines are largely based on pediatric clinical trials and retrospective series. Limited data exist with respect to clinical characteristics, prognostic factors, and outcomes based on first-line treatments. METHODS: Two hundred adults with MB seen at a single institution from January 1978 to April 2017 were identified and followed for a median of 8.4 y (7.1, 10.3). RESULTS: Patient’s median age at diagnosis was 29 y (18, 63). One hundred eleven (55.5%) were standard-risk, 59 (29.5%) were high-risk, and 30 (15.0%) were indeterminate. Most received post-operative radiation (RT) (184 [92.0%]), and 105 (52.5%) received first-line chemotherapy. Median overall survival (OS) was 8.8 y (7.2, 12.2) and median progression-free survival (PFS) was 6.6 y (4.9, 11.2). High-risk patients had inferior OS (Hazard ratio [HR] = 2.5 [1.5, 4.2], P = .0006) and PFS (HR = 2.3 [1.3, 3.9], P = .002) compared to standard-risk patients. Age, sex, and metastatic disease were not associated with survival. After adjusting for risk status, those who received RT plus adjuvant chemotherapy had superior PFS compared to RT plus neoadjuvant chemotherapy [HR = 0.46 (0.22, 0.95), P = .0357]. Within a subgroup for whom detailed clinical data were available, those who received RT plus adjuvant chemotherapy had improved PFS compared to RT only [HR = 0.24 (0.074–0.76), P = .016]. The substitution of cisplatin for carboplatin and the elimination of vincristine did not negatively affect outcomes. CONCLUSION: This is the largest single-institution retrospective study of adult MB to our knowledge and identifies standard-risk status, first-line RT and adjuvant chemotherapy as factors associated with improved outcomes. |
format | Online Article Text |
id | pubmed-8350154 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-83501542021-08-09 Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience Majd, Nazanin K Mastall, Maximilan Lin, Heather Dibaj, Seyede Shiva Hess, Kenneth R Yuan, Ying Garcia, Manuela Martin-Bejarano Fuller, Gregory N Alfaro, Kristin D Gule-Monroe, Maria K Huse, Jason T Khatua, Soumen Rao, Ganesh Sandberg, David I Wefel, Jeffrey S Yeboa, Debra N Paulino, Arnold C McGovern, Susan L Zaky, Wafik Mahajan, Anita Suki, Dima Weathers, Shiao-Pei Harriso, Rebecca A De Groo, John F Puduvalli, Vinay K Penas-Prado, Marta Neurooncol Adv Clinical Investigations BACKGROUND: Adult medulloblastoma (MB) is rare, and management guidelines are largely based on pediatric clinical trials and retrospective series. Limited data exist with respect to clinical characteristics, prognostic factors, and outcomes based on first-line treatments. METHODS: Two hundred adults with MB seen at a single institution from January 1978 to April 2017 were identified and followed for a median of 8.4 y (7.1, 10.3). RESULTS: Patient’s median age at diagnosis was 29 y (18, 63). One hundred eleven (55.5%) were standard-risk, 59 (29.5%) were high-risk, and 30 (15.0%) were indeterminate. Most received post-operative radiation (RT) (184 [92.0%]), and 105 (52.5%) received first-line chemotherapy. Median overall survival (OS) was 8.8 y (7.2, 12.2) and median progression-free survival (PFS) was 6.6 y (4.9, 11.2). High-risk patients had inferior OS (Hazard ratio [HR] = 2.5 [1.5, 4.2], P = .0006) and PFS (HR = 2.3 [1.3, 3.9], P = .002) compared to standard-risk patients. Age, sex, and metastatic disease were not associated with survival. After adjusting for risk status, those who received RT plus adjuvant chemotherapy had superior PFS compared to RT plus neoadjuvant chemotherapy [HR = 0.46 (0.22, 0.95), P = .0357]. Within a subgroup for whom detailed clinical data were available, those who received RT plus adjuvant chemotherapy had improved PFS compared to RT only [HR = 0.24 (0.074–0.76), P = .016]. The substitution of cisplatin for carboplatin and the elimination of vincristine did not negatively affect outcomes. CONCLUSION: This is the largest single-institution retrospective study of adult MB to our knowledge and identifies standard-risk status, first-line RT and adjuvant chemotherapy as factors associated with improved outcomes. Oxford University Press 2021-06-22 /pmc/articles/PMC8350154/ /pubmed/34377987 http://dx.doi.org/10.1093/noajnl/vdab079 Text en © The Author(s) 2021. 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 (http://creativecommons.org/licenses/by-nc/4.0/ (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 | Clinical Investigations Majd, Nazanin K Mastall, Maximilan Lin, Heather Dibaj, Seyede Shiva Hess, Kenneth R Yuan, Ying Garcia, Manuela Martin-Bejarano Fuller, Gregory N Alfaro, Kristin D Gule-Monroe, Maria K Huse, Jason T Khatua, Soumen Rao, Ganesh Sandberg, David I Wefel, Jeffrey S Yeboa, Debra N Paulino, Arnold C McGovern, Susan L Zaky, Wafik Mahajan, Anita Suki, Dima Weathers, Shiao-Pei Harriso, Rebecca A De Groo, John F Puduvalli, Vinay K Penas-Prado, Marta Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience |
title | Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience |
title_full | Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience |
title_fullStr | Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience |
title_full_unstemmed | Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience |
title_short | Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience |
title_sort | clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; the md anderson cancer center experience |
topic | Clinical Investigations |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8350154/ https://www.ncbi.nlm.nih.gov/pubmed/34377987 http://dx.doi.org/10.1093/noajnl/vdab079 |
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