Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature

Sclerosing epithelioid fibrosarcoma (SEF) is a rare subtype of soft tissue tumors, and SEF originating from the side of the spine is even rarer. We report that a 28-year-old young woman suffered from chest pain and back pain for 3 years, and thereafter she went to see a doctor because her condition...

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Main Authors: Changhong Wei, Yili Ma, Dengqiang Wu, Xiaoyu Chen, Chunjun Li, Jun Chen, Sufang Zhou
Format: Article
Language:English
Published: Frontiers Media S.A. 2022-06-01
Series:Frontiers in Medicine
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fmed.2022.833864/full
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author Changhong Wei
Changhong Wei
Yili Ma
Dengqiang Wu
Xiaoyu Chen
Chunjun Li
Jun Chen
Sufang Zhou
Sufang Zhou
author_facet Changhong Wei
Changhong Wei
Yili Ma
Dengqiang Wu
Xiaoyu Chen
Chunjun Li
Jun Chen
Sufang Zhou
Sufang Zhou
author_sort Changhong Wei
collection DOAJ
description Sclerosing epithelioid fibrosarcoma (SEF) is a rare subtype of soft tissue tumors, and SEF originating from the side of the spine is even rarer. We report that a 28-year-old young woman suffered from chest pain and back pain for 3 years, and thereafter she went to see a doctor because her condition deteriorated. Enhanced CT showed that the right posterior upper chest wall mass invaded the adjacent bone, and the boundary between the lesion and the surrounding tissues was relatively clear. She then underwent posterior tumor removal surgery. The pathological examination confirmed the diagnosis of SEF. In histomorphology, the tumor displayed a typical epithelioid clear cell morphology, accompanied by extensive vitrification and fibrosis, which better helped to differentiate the tumor from low grade fibromyxoid sarcoma, solitary fibrous tumor and other entities. The immunohistochemical analysis showed a diffuse positive reaction to MUC4, a highly specific marker of SEF, which was detected by Immunohistochemistry (IHC), and fluorescence in-situ hybridization (FISH) confirmed that the EWSR1 gene was rearranged, while the FUS gene was not rearranged. This is the first time that we have encountered such this rare case and thus report this case with updated literature related to this tumor.
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spelling doaj.art-a126185ad00c47d397715593972225572022-12-22T03:33:21ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2022-06-01910.3389/fmed.2022.833864833864Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of LiteratureChanghong Wei0Changhong Wei1Yili Ma2Dengqiang Wu3Xiaoyu Chen4Chunjun Li5Jun Chen6Sufang Zhou7Sufang Zhou8Department of Pathology, Guangxi Medical University Cancer Hospital, Nanning, ChinaGuangxi Key Laboratory of Bio-Targeting Theranostics, National Center for International Research of Bio-Targeting Theranostics, Collaborative Innovation Center for Targeting Tumor Diagnosis and Therapy, Guangxi Medical University, Nanning, ChinaDepartment of Biochemistry and Molecular Biology, School of Pre-clinical Science, Guangxi Medical University, Nanning, ChinaGuangxi Key Laboratory of Bio-Targeting Theranostics, National Center for International Research of Bio-Targeting Theranostics, Collaborative Innovation Center for Targeting Tumor Diagnosis and Therapy, Guangxi Medical University, Nanning, ChinaDepartment of Pathology, Guangxi Medical University Cancer Hospital, Nanning, ChinaDepartment of Pathology, Guangxi Medical University Cancer Hospital, Nanning, ChinaDepartment of Pathology, Guangxi Medical University Cancer Hospital, Nanning, ChinaGuangxi Key Laboratory of Bio-Targeting Theranostics, National Center for International Research of Bio-Targeting Theranostics, Collaborative Innovation Center for Targeting Tumor Diagnosis and Therapy, Guangxi Medical University, Nanning, ChinaDepartment of Biochemistry and Molecular Biology, School of Pre-clinical Science, Guangxi Medical University, Nanning, ChinaSclerosing epithelioid fibrosarcoma (SEF) is a rare subtype of soft tissue tumors, and SEF originating from the side of the spine is even rarer. We report that a 28-year-old young woman suffered from chest pain and back pain for 3 years, and thereafter she went to see a doctor because her condition deteriorated. Enhanced CT showed that the right posterior upper chest wall mass invaded the adjacent bone, and the boundary between the lesion and the surrounding tissues was relatively clear. She then underwent posterior tumor removal surgery. The pathological examination confirmed the diagnosis of SEF. In histomorphology, the tumor displayed a typical epithelioid clear cell morphology, accompanied by extensive vitrification and fibrosis, which better helped to differentiate the tumor from low grade fibromyxoid sarcoma, solitary fibrous tumor and other entities. The immunohistochemical analysis showed a diffuse positive reaction to MUC4, a highly specific marker of SEF, which was detected by Immunohistochemistry (IHC), and fluorescence in-situ hybridization (FISH) confirmed that the EWSR1 gene was rearranged, while the FUS gene was not rearranged. This is the first time that we have encountered such this rare case and thus report this case with updated literature related to this tumor.https://www.frontiersin.org/articles/10.3389/fmed.2022.833864/fullsoft tissue sarcomasclerosing epithelioid fibrosarcomaimmunohistochemistryfishEWSR1
spellingShingle Changhong Wei
Changhong Wei
Yili Ma
Dengqiang Wu
Xiaoyu Chen
Chunjun Li
Jun Chen
Sufang Zhou
Sufang Zhou
Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature
Frontiers in Medicine
soft tissue sarcoma
sclerosing epithelioid fibrosarcoma
immunohistochemistry
fish
EWSR1
title Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature
title_full Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature
title_fullStr Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature
title_full_unstemmed Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature
title_short Sclerosing Epithelioid Fibrosarcoma of the Thoracic Vertebrae: An Fairly Unusual Case Report With a Short Review of Literature
title_sort sclerosing epithelioid fibrosarcoma of the thoracic vertebrae an fairly unusual case report with a short review of literature
topic soft tissue sarcoma
sclerosing epithelioid fibrosarcoma
immunohistochemistry
fish
EWSR1
url https://www.frontiersin.org/articles/10.3389/fmed.2022.833864/full
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