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Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas

BACKGROUND: Chordomas are rare malignancies that primarily affect adults, but also rarely affect pediatric patients. We compared the imaging appearance, demographic and anatomic distributions of adult and pediatric chordomas in a large cohort. METHODS: We performed a retrospective review of medical...

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Autores principales: Sebro, Ronnie, DeLaney, Thomas, Hornicek, Francis, Schwab, Joseph, Choy, Edwin, Nielsen, G. Petur, Rosenthal, Daniel I.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5016865/
https://www.ncbi.nlm.nih.gov/pubmed/27609115
http://dx.doi.org/10.1186/s12880-016-0149-5
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author Sebro, Ronnie
DeLaney, Thomas
Hornicek, Francis
Schwab, Joseph
Choy, Edwin
Nielsen, G. Petur
Rosenthal, Daniel I.
author_facet Sebro, Ronnie
DeLaney, Thomas
Hornicek, Francis
Schwab, Joseph
Choy, Edwin
Nielsen, G. Petur
Rosenthal, Daniel I.
author_sort Sebro, Ronnie
collection PubMed
description BACKGROUND: Chordomas are rare malignancies that primarily affect adults, but also rarely affect pediatric patients. We compared the imaging appearance, demographic and anatomic distributions of adult and pediatric chordomas in a large cohort. METHODS: We performed a retrospective review of medical records of 220 subjects with histologically confirmed chordomas of the axial skeleton and pre-treatment magnetic resonance imaging studies. Age, sex, type of chordoma (conventional, chondroid or dedifferentiated), the anatomic location of the chordoma, as well as whether the lesion was primarily extra-osseous were recorded. Pediatric subjects were less than 21 years at the time of diagnosis. Binomial two-sample tests of proportions and Fisher’s exact tests were used to compare proportions between the pediatric and adult subjects. RESULTS: Fifty six pediatric subjects (58.9 % female) and 164 adult subjects (42.1 % female) were identified. The proportion of female subjects with chordomas was significantly higher in the pediatric cohort compared to the adult cohort (P = 0.04). Most chordomas occur in Caucasians, however African-Americans were more represented in the pediatric cohort than in the adult cohort (P = 0.01). 69.6 % (39/56) of the pediatric chordomas involved the clivus/skull base and cervical spine compared to 29.3 % (48/164) of the adult chordomas (P = 1.99 × 10(−7)). Only 1.8 % (1/56) of the pediatric chordomas was in the sacrococcygeal region compared to 36.0 % (59/164) of the adult chordomas (P = 2.55 × 10(−8)). In cases where pre-treatment imaging was available, 93.8 % (16/17) of pediatric chordomas were predominantly extra-osseous compared to 76.7 % (46/60) of adult chordomas (P = 0.17). CONCLUSIONS: Pediatric chordomas more often affect females and occur most frequently at the craniocervical junction with decrease in incidence distally in the spine, whereas adult chordomas most frequently involve the craniocervical and sacrococcygeal regions.
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spelling pubmed-50168652016-09-10 Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas Sebro, Ronnie DeLaney, Thomas Hornicek, Francis Schwab, Joseph Choy, Edwin Nielsen, G. Petur Rosenthal, Daniel I. BMC Med Imaging Research Article BACKGROUND: Chordomas are rare malignancies that primarily affect adults, but also rarely affect pediatric patients. We compared the imaging appearance, demographic and anatomic distributions of adult and pediatric chordomas in a large cohort. METHODS: We performed a retrospective review of medical records of 220 subjects with histologically confirmed chordomas of the axial skeleton and pre-treatment magnetic resonance imaging studies. Age, sex, type of chordoma (conventional, chondroid or dedifferentiated), the anatomic location of the chordoma, as well as whether the lesion was primarily extra-osseous were recorded. Pediatric subjects were less than 21 years at the time of diagnosis. Binomial two-sample tests of proportions and Fisher’s exact tests were used to compare proportions between the pediatric and adult subjects. RESULTS: Fifty six pediatric subjects (58.9 % female) and 164 adult subjects (42.1 % female) were identified. The proportion of female subjects with chordomas was significantly higher in the pediatric cohort compared to the adult cohort (P = 0.04). Most chordomas occur in Caucasians, however African-Americans were more represented in the pediatric cohort than in the adult cohort (P = 0.01). 69.6 % (39/56) of the pediatric chordomas involved the clivus/skull base and cervical spine compared to 29.3 % (48/164) of the adult chordomas (P = 1.99 × 10(−7)). Only 1.8 % (1/56) of the pediatric chordomas was in the sacrococcygeal region compared to 36.0 % (59/164) of the adult chordomas (P = 2.55 × 10(−8)). In cases where pre-treatment imaging was available, 93.8 % (16/17) of pediatric chordomas were predominantly extra-osseous compared to 76.7 % (46/60) of adult chordomas (P = 0.17). CONCLUSIONS: Pediatric chordomas more often affect females and occur most frequently at the craniocervical junction with decrease in incidence distally in the spine, whereas adult chordomas most frequently involve the craniocervical and sacrococcygeal regions. BioMed Central 2016-09-08 /pmc/articles/PMC5016865/ /pubmed/27609115 http://dx.doi.org/10.1186/s12880-016-0149-5 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Sebro, Ronnie
DeLaney, Thomas
Hornicek, Francis
Schwab, Joseph
Choy, Edwin
Nielsen, G. Petur
Rosenthal, Daniel I.
Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas
title Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas
title_full Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas
title_fullStr Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas
title_full_unstemmed Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas
title_short Differences in sex distribution, anatomic location and MR imaging appearance of pediatric compared to adult chordomas
title_sort differences in sex distribution, anatomic location and mr imaging appearance of pediatric compared to adult chordomas
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5016865/
https://www.ncbi.nlm.nih.gov/pubmed/27609115
http://dx.doi.org/10.1186/s12880-016-0149-5
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