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Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child

Primary extraskeletal Ewing sarcoma (EES) is a rare small round cell malignancy that accounts for less than 1% of all sarcomas. It is found most commonly in the trunk and lower limbs and very rarely in the pleura and can be easily misdiagnosed in clinical practice. This study presents the case of an...

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Autores principales: Wu, Yang, Xie, Chao-Bang, Huang, Qiong, Zhao, Kai-Fei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10102462/
https://www.ncbi.nlm.nih.gov/pubmed/37064103
http://dx.doi.org/10.3389/fonc.2023.1137586
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author Wu, Yang
Xie, Chao-Bang
Huang, Qiong
Zhao, Kai-Fei
author_facet Wu, Yang
Xie, Chao-Bang
Huang, Qiong
Zhao, Kai-Fei
author_sort Wu, Yang
collection PubMed
description Primary extraskeletal Ewing sarcoma (EES) is a rare small round cell malignancy that accounts for less than 1% of all sarcomas. It is found most commonly in the trunk and lower limbs and very rarely in the pleura and can be easily misdiagnosed in clinical practice. This study presents the case of an 11-year-old boy who presented to our hospital with no apparent cause of left shoulder pain for 6 months. On physical examination, tenderness was noted in the left chest wall and shoulder joint, which had a limited range of motion. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest revealed an irregular soft tissue mass in the upper left thorax, with a wide base attached to the adjacent pleura and bone destruction of the adjacent left first rib. The patient’s bone scan showed a dense focus of increased radiotracer accumulation in the left first rib. A subsequent CT-guided aspiration biopsy of the left pleural mass with histomorphology and immunohistochemical phenotyping led to a diagnosis of extraskeletal Ewing sarcoma. To inhibit tumor growth, alternating systemic chemotherapy cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) and isocyclophosphamide and etoposide (IE) were administered at 3-week intervals. After completing three VDC and two IE cycles, the child’s condition was well and the pain in the left shoulder joint was relieved. However, a repeat MRI of the chest showed that the mass did not shrink.
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spelling pubmed-101024622023-04-15 Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child Wu, Yang Xie, Chao-Bang Huang, Qiong Zhao, Kai-Fei Front Oncol Oncology Primary extraskeletal Ewing sarcoma (EES) is a rare small round cell malignancy that accounts for less than 1% of all sarcomas. It is found most commonly in the trunk and lower limbs and very rarely in the pleura and can be easily misdiagnosed in clinical practice. This study presents the case of an 11-year-old boy who presented to our hospital with no apparent cause of left shoulder pain for 6 months. On physical examination, tenderness was noted in the left chest wall and shoulder joint, which had a limited range of motion. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest revealed an irregular soft tissue mass in the upper left thorax, with a wide base attached to the adjacent pleura and bone destruction of the adjacent left first rib. The patient’s bone scan showed a dense focus of increased radiotracer accumulation in the left first rib. A subsequent CT-guided aspiration biopsy of the left pleural mass with histomorphology and immunohistochemical phenotyping led to a diagnosis of extraskeletal Ewing sarcoma. To inhibit tumor growth, alternating systemic chemotherapy cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) and isocyclophosphamide and etoposide (IE) were administered at 3-week intervals. After completing three VDC and two IE cycles, the child’s condition was well and the pain in the left shoulder joint was relieved. However, a repeat MRI of the chest showed that the mass did not shrink. Frontiers Media S.A. 2023-03-31 /pmc/articles/PMC10102462/ /pubmed/37064103 http://dx.doi.org/10.3389/fonc.2023.1137586 Text en Copyright © 2023 Wu, Xie, Huang and Zhao https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Wu, Yang
Xie, Chao-Bang
Huang, Qiong
Zhao, Kai-Fei
Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child
title Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child
title_full Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child
title_fullStr Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child
title_full_unstemmed Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child
title_short Case report: Primary pleural giant extraskeletal Ewing sarcoma in a child
title_sort case report: primary pleural giant extraskeletal ewing sarcoma in a child
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10102462/
https://www.ncbi.nlm.nih.gov/pubmed/37064103
http://dx.doi.org/10.3389/fonc.2023.1137586
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