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Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival
BACKGROUND: Secondary peripheral chondrosarcomas arising in solitary osteochondromas is an unusual complication, reported in small series. In this study, we aimed to present our experience with this rare variant of chondrosarcoma and compare results with already published data in order to determine...
Autores principales: | , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8864784/ https://www.ncbi.nlm.nih.gov/pubmed/35193636 http://dx.doi.org/10.1186/s13023-022-02210-2 |
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author | Righi, Alberto Pacheco, Marina Cocchi, Stefania Asioli, Sofia Gambarotti, Marco Donati, Davide Maria Evangelista, Andrea Gnoli, Maria Locatelli, Manuela Mordenti, Marina Boarini, Manila Brizola, Evelise Pedrini, Elena Sangiorgi, Luca |
author_facet | Righi, Alberto Pacheco, Marina Cocchi, Stefania Asioli, Sofia Gambarotti, Marco Donati, Davide Maria Evangelista, Andrea Gnoli, Maria Locatelli, Manuela Mordenti, Marina Boarini, Manila Brizola, Evelise Pedrini, Elena Sangiorgi, Luca |
author_sort | Righi, Alberto |
collection | PubMed |
description | BACKGROUND: Secondary peripheral chondrosarcomas arising in solitary osteochondromas is an unusual complication, reported in small series. In this study, we aimed to present our experience with this rare variant of chondrosarcoma and compare results with already published data in order to determine prognostic factors for overall and disease-free survival. METHODS: The case study includes retrospective data from patients diagnosed at a single institution from 1943 to 2019. Clinical data were collected reviewing all available medical records from first to last follow-up visits. To exclude the presence of the Multiple Osteochondroma Hereditary Syndrome, few patients, with a suspect of a familial form of the disease, were evaluated for the presence of germline heterozygous variants in EXT1 and EXT2 genes. Results were summarized using descriptive statistics and statistical analysis were performed to reveal associations between variables. RESULTS: Two hundred and fourteen secondary peripheral chondrosarcomas that arose exclusively from solitary osteochondromas diagnosed in a multidisciplinary setting at the IRCCS Istituto Ortopedico Rizzoli were retrospectively identified, 66.4% males and 33.6% females with a median age at diagnosis of 38 years. The local recurrence rate was 17.3%, while the metastases one was 5.1%. Besides age, a high histologic grade is the only factor associated with worse 5-year and 10-year overall survival (log-rank p = 0.0005, HR = 3.74; 95% CI 1.69–8.26). Moreover, high histological grade (HR = 3.75; 95% CI = 1.69–8.34; p = 0.001) and surgical debulking (HR = 3.71; 95% CI = 1.57–8.79; p = 0.003) were associated with a significantly worse disease-free survival. CONCLUSIONS: Our study confirm the low-grade behavior of secondary peripheral chondrosarcomas and demonstrate that the best choice of treatment for those arising in solitary osteochondromas is the wide surgical excision, when possible. Location per se is not a factor that affects prognosis, while the accurate histological grade assessment is correlated with the tumor aggressiveness and a long term follow up is necessary for this rare variant of chondrosarcoma. |
format | Online Article Text |
id | pubmed-8864784 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-88647842022-02-23 Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival Righi, Alberto Pacheco, Marina Cocchi, Stefania Asioli, Sofia Gambarotti, Marco Donati, Davide Maria Evangelista, Andrea Gnoli, Maria Locatelli, Manuela Mordenti, Marina Boarini, Manila Brizola, Evelise Pedrini, Elena Sangiorgi, Luca Orphanet J Rare Dis Research BACKGROUND: Secondary peripheral chondrosarcomas arising in solitary osteochondromas is an unusual complication, reported in small series. In this study, we aimed to present our experience with this rare variant of chondrosarcoma and compare results with already published data in order to determine prognostic factors for overall and disease-free survival. METHODS: The case study includes retrospective data from patients diagnosed at a single institution from 1943 to 2019. Clinical data were collected reviewing all available medical records from first to last follow-up visits. To exclude the presence of the Multiple Osteochondroma Hereditary Syndrome, few patients, with a suspect of a familial form of the disease, were evaluated for the presence of germline heterozygous variants in EXT1 and EXT2 genes. Results were summarized using descriptive statistics and statistical analysis were performed to reveal associations between variables. RESULTS: Two hundred and fourteen secondary peripheral chondrosarcomas that arose exclusively from solitary osteochondromas diagnosed in a multidisciplinary setting at the IRCCS Istituto Ortopedico Rizzoli were retrospectively identified, 66.4% males and 33.6% females with a median age at diagnosis of 38 years. The local recurrence rate was 17.3%, while the metastases one was 5.1%. Besides age, a high histologic grade is the only factor associated with worse 5-year and 10-year overall survival (log-rank p = 0.0005, HR = 3.74; 95% CI 1.69–8.26). Moreover, high histological grade (HR = 3.75; 95% CI = 1.69–8.34; p = 0.001) and surgical debulking (HR = 3.71; 95% CI = 1.57–8.79; p = 0.003) were associated with a significantly worse disease-free survival. CONCLUSIONS: Our study confirm the low-grade behavior of secondary peripheral chondrosarcomas and demonstrate that the best choice of treatment for those arising in solitary osteochondromas is the wide surgical excision, when possible. Location per se is not a factor that affects prognosis, while the accurate histological grade assessment is correlated with the tumor aggressiveness and a long term follow up is necessary for this rare variant of chondrosarcoma. BioMed Central 2022-02-22 /pmc/articles/PMC8864784/ /pubmed/35193636 http://dx.doi.org/10.1186/s13023-022-02210-2 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Righi, Alberto Pacheco, Marina Cocchi, Stefania Asioli, Sofia Gambarotti, Marco Donati, Davide Maria Evangelista, Andrea Gnoli, Maria Locatelli, Manuela Mordenti, Marina Boarini, Manila Brizola, Evelise Pedrini, Elena Sangiorgi, Luca Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
title | Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
title_full | Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
title_fullStr | Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
title_full_unstemmed | Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
title_short | Secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
title_sort | secondary peripheral chondrosarcoma arising in solitary osteochondroma: variables influencing prognosis and survival |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8864784/ https://www.ncbi.nlm.nih.gov/pubmed/35193636 http://dx.doi.org/10.1186/s13023-022-02210-2 |
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