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RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS
Pediatric chordomas are exceedingly rare and there are limited data to guide treatment decisions. We report a retrospective analysis of 19 patients with chordomas who received treatment at our institution from 2001–2020. Of the 19 patients, 15 had clival (79%), 3 cervical and 1 sacral chordoma. Ther...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7715682/ http://dx.doi.org/10.1093/neuonc/noaa222.754 |
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author | Gibson, Amber Baig, Muhammad Raza, Shaan Grosshans, David Zaky, Wafik |
author_facet | Gibson, Amber Baig, Muhammad Raza, Shaan Grosshans, David Zaky, Wafik |
author_sort | Gibson, Amber |
collection | PubMed |
description | Pediatric chordomas are exceedingly rare and there are limited data to guide treatment decisions. We report a retrospective analysis of 19 patients with chordomas who received treatment at our institution from 2001–2020. Of the 19 patients, 15 had clival (79%), 3 cervical and 1 sacral chordoma. There were 9 males (47%). Median age at diagnosis of 10.6 years. Eight patients had gross total (42%) and 11 (58%) had sub-total resection. As front line therapy 15 patients (79%) underwent surgery followed by radiation (1 photon and 14 proton), 3 patients (16%) received surgery and chemotherapy (anaplastic histology) and 1 patient received only surgery. For patients treated with radiation therapy the average prescribed dose was 70 Gy (range: 52–74). Post-surgery and radiation, 14 of 15 patients remained in remission. Five (26%) patients had progressive disease (PD) with median time to progression of 13 months of whom 3 died of disease at median of 18 months. Treatment of PD consisted of chemotherapy and radiation for 3, re-resection with radiation for 1 and chemotherapy alone for 1 patient. The patients with metastatic and anaplastic disease mean survival is 21 month versus 45 for the rest of the cohort. In summary, post-operative adjuvant radiation provided an overall good outcome in majority of patients. Patients with anaplastic pathology and metastasis at diagnosis had worse outcome. Those who relapsed, subsequent treatment was palliative at best with short survival. Molecular analysis is warranted in future for better disease stratification. |
format | Online Article Text |
id | pubmed-7715682 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-77156822020-12-09 RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS Gibson, Amber Baig, Muhammad Raza, Shaan Grosshans, David Zaky, Wafik Neuro Oncol Craniopharyngioma and Rare Tumors Pediatric chordomas are exceedingly rare and there are limited data to guide treatment decisions. We report a retrospective analysis of 19 patients with chordomas who received treatment at our institution from 2001–2020. Of the 19 patients, 15 had clival (79%), 3 cervical and 1 sacral chordoma. There were 9 males (47%). Median age at diagnosis of 10.6 years. Eight patients had gross total (42%) and 11 (58%) had sub-total resection. As front line therapy 15 patients (79%) underwent surgery followed by radiation (1 photon and 14 proton), 3 patients (16%) received surgery and chemotherapy (anaplastic histology) and 1 patient received only surgery. For patients treated with radiation therapy the average prescribed dose was 70 Gy (range: 52–74). Post-surgery and radiation, 14 of 15 patients remained in remission. Five (26%) patients had progressive disease (PD) with median time to progression of 13 months of whom 3 died of disease at median of 18 months. Treatment of PD consisted of chemotherapy and radiation for 3, re-resection with radiation for 1 and chemotherapy alone for 1 patient. The patients with metastatic and anaplastic disease mean survival is 21 month versus 45 for the rest of the cohort. In summary, post-operative adjuvant radiation provided an overall good outcome in majority of patients. Patients with anaplastic pathology and metastasis at diagnosis had worse outcome. Those who relapsed, subsequent treatment was palliative at best with short survival. Molecular analysis is warranted in future for better disease stratification. Oxford University Press 2020-12-04 /pmc/articles/PMC7715682/ http://dx.doi.org/10.1093/neuonc/noaa222.754 Text en © The Author(s) 2020. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://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 | Craniopharyngioma and Rare Tumors Gibson, Amber Baig, Muhammad Raza, Shaan Grosshans, David Zaky, Wafik RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS |
title | RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS |
title_full | RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS |
title_fullStr | RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS |
title_full_unstemmed | RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS |
title_short | RARE-44. CLINICAL CHARACTERIZATION AND OUTCOME; OUR EXPERIENCE OF CHORDOMAS IN PEDIATRIC AND YOUNG ADULTS |
title_sort | rare-44. clinical characterization and outcome; our experience of chordomas in pediatric and young adults |
topic | Craniopharyngioma and Rare Tumors |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7715682/ http://dx.doi.org/10.1093/neuonc/noaa222.754 |
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