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Extraskeletal Osteosarcoma: An International Rare Cancer Network Study

OBJECTIVES: To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS). MATERIALS AND METHODS: Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treat...

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Autores principales: Sio, Terence T., Vu, Charles C., Sohawon, Schoeb, Van Houtte, Paul, Thariat, Juliette, Novotny, Paul J., Miller, Robert C., Bar-Sela, Gil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4892767/
https://www.ncbi.nlm.nih.gov/pubmed/24401667
http://dx.doi.org/10.1097/COC.0000000000000005
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author Sio, Terence T.
Vu, Charles C.
Sohawon, Schoeb
Van Houtte, Paul
Thariat, Juliette
Novotny, Paul J.
Miller, Robert C.
Bar-Sela, Gil
author_facet Sio, Terence T.
Vu, Charles C.
Sohawon, Schoeb
Van Houtte, Paul
Thariat, Juliette
Novotny, Paul J.
Miller, Robert C.
Bar-Sela, Gil
author_sort Sio, Terence T.
collection PubMed
description OBJECTIVES: To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS). MATERIALS AND METHODS: Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treatment information, and survival status were analyzed. RESULTS: Thirty-seven patients in 4 health care institutions were identified. Thirty-one (86%) patients had grade 3 or 4 tumors. Most patients (27 [73%]) had stage III disease. Fourteen (38%) received neoadjuvant chemotherapy or chemoradiation. Of 28 (85%) who underwent surgery, 21 (75%) had free margins achieved and 15 (41%) subsequently received adjuvant chemotherapy. At median follow-up of 45 months, 20 (55%) patients were alive, 13 (43%) of whom were disease free. Univariate analysis showed that poor overall survival was related to stage IV (P<0.001), no surgery (P<0.001), primary size >10 cm (P=0.002), and age (P=0.002). In multivariate analysis, primary size >10 cm (P=0.005) was prognostic for overall survival. For patients without metastases, univariate analysis showed disease-free survival (DFS) related to primary size >10 cm (P=0.003), surgery (P=0.004), local recurrence (P=0.003), and age (P<0.001). In multivariate analysis for DFS, primary size >10 cm (P=0.01) and older age (P<0.001) were significant for worse outcome. CONCLUSIONS: Multimodality treatment remains standard for localized ESOS, with indications for neoadjuvant therapy less clear. Larger tumor size and older age were prognostic of poorer DFS.
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spelling pubmed-48927672016-06-21 Extraskeletal Osteosarcoma: An International Rare Cancer Network Study Sio, Terence T. Vu, Charles C. Sohawon, Schoeb Van Houtte, Paul Thariat, Juliette Novotny, Paul J. Miller, Robert C. Bar-Sela, Gil Am J Clin Oncol Original Articles: Soft Tissue OBJECTIVES: To report characteristics, treatment, and outcomes for an international cohort of patients with extraskeletal osteosarcoma (ESOS). MATERIALS AND METHODS: Through the Rare Cancer Network, retrospective data on patients with ESOS were collected. Patient characteristics, multimodality treatment information, and survival status were analyzed. RESULTS: Thirty-seven patients in 4 health care institutions were identified. Thirty-one (86%) patients had grade 3 or 4 tumors. Most patients (27 [73%]) had stage III disease. Fourteen (38%) received neoadjuvant chemotherapy or chemoradiation. Of 28 (85%) who underwent surgery, 21 (75%) had free margins achieved and 15 (41%) subsequently received adjuvant chemotherapy. At median follow-up of 45 months, 20 (55%) patients were alive, 13 (43%) of whom were disease free. Univariate analysis showed that poor overall survival was related to stage IV (P<0.001), no surgery (P<0.001), primary size >10 cm (P=0.002), and age (P=0.002). In multivariate analysis, primary size >10 cm (P=0.005) was prognostic for overall survival. For patients without metastases, univariate analysis showed disease-free survival (DFS) related to primary size >10 cm (P=0.003), surgery (P=0.004), local recurrence (P=0.003), and age (P<0.001). In multivariate analysis for DFS, primary size >10 cm (P=0.01) and older age (P<0.001) were significant for worse outcome. CONCLUSIONS: Multimodality treatment remains standard for localized ESOS, with indications for neoadjuvant therapy less clear. Larger tumor size and older age were prognostic of poorer DFS. Lippincott Williams & Wilkins 2016-02 2016-02-03 /pmc/articles/PMC4892767/ /pubmed/24401667 http://dx.doi.org/10.1097/COC.0000000000000005 Text en Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially. http://creativecommons.org/licenses/by-nc-nd/4.0/.
spellingShingle Original Articles: Soft Tissue
Sio, Terence T.
Vu, Charles C.
Sohawon, Schoeb
Van Houtte, Paul
Thariat, Juliette
Novotny, Paul J.
Miller, Robert C.
Bar-Sela, Gil
Extraskeletal Osteosarcoma: An International Rare Cancer Network Study
title Extraskeletal Osteosarcoma: An International Rare Cancer Network Study
title_full Extraskeletal Osteosarcoma: An International Rare Cancer Network Study
title_fullStr Extraskeletal Osteosarcoma: An International Rare Cancer Network Study
title_full_unstemmed Extraskeletal Osteosarcoma: An International Rare Cancer Network Study
title_short Extraskeletal Osteosarcoma: An International Rare Cancer Network Study
title_sort extraskeletal osteosarcoma: an international rare cancer network study
topic Original Articles: Soft Tissue
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4892767/
https://www.ncbi.nlm.nih.gov/pubmed/24401667
http://dx.doi.org/10.1097/COC.0000000000000005
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