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Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report

Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. Primary epithelioid sarcoma arising from the sp...

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Autores principales: Tan, Yi Liang, Ong, Wilson, Tan, Jiong Hao, Kumar, Naresh, Hallinan, James Thomas Patrick Decourcy
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10488709/
https://www.ncbi.nlm.nih.gov/pubmed/37685699
http://dx.doi.org/10.3390/jcm12175632
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author Tan, Yi Liang
Ong, Wilson
Tan, Jiong Hao
Kumar, Naresh
Hallinan, James Thomas Patrick Decourcy
author_facet Tan, Yi Liang
Ong, Wilson
Tan, Jiong Hao
Kumar, Naresh
Hallinan, James Thomas Patrick Decourcy
author_sort Tan, Yi Liang
collection PubMed
description Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. Primary epithelioid sarcoma arising from the spine is extremely rare, with limited data in the literature. We review the existing literature regarding spinal epithelioid sarcoma and report a case of epithelioid sarcoma arising from the spinal cord. A 54 year old male presented with a 1-month history of progressive left upper-limb weakness and numbness. Magnetic resonance imaging (MRI) of the spine showed an enhancing intramedullary mass at the level of T1 also involving the left T1 nerve root. Systemic radiological examination revealed no other lesion at presentation. Surgical excision of the mass was performed, and histology was consistent with epithelioid sarcoma of the spine. Despite adjuvant radiotherapy, there was aggressive local recurrence and development of intracranial metastatic spread. The patient died of the disease within 5 months from presentation. To the best of our knowledge, spinal epithelioid sarcoma arising from the spinal cord has not yet been reported. We review the challenges in diagnosis, surgical treatment, and oncologic outcome of this case.
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spelling pubmed-104887092023-09-09 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report Tan, Yi Liang Ong, Wilson Tan, Jiong Hao Kumar, Naresh Hallinan, James Thomas Patrick Decourcy J Clin Med Case Report Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. Primary epithelioid sarcoma arising from the spine is extremely rare, with limited data in the literature. We review the existing literature regarding spinal epithelioid sarcoma and report a case of epithelioid sarcoma arising from the spinal cord. A 54 year old male presented with a 1-month history of progressive left upper-limb weakness and numbness. Magnetic resonance imaging (MRI) of the spine showed an enhancing intramedullary mass at the level of T1 also involving the left T1 nerve root. Systemic radiological examination revealed no other lesion at presentation. Surgical excision of the mass was performed, and histology was consistent with epithelioid sarcoma of the spine. Despite adjuvant radiotherapy, there was aggressive local recurrence and development of intracranial metastatic spread. The patient died of the disease within 5 months from presentation. To the best of our knowledge, spinal epithelioid sarcoma arising from the spinal cord has not yet been reported. We review the challenges in diagnosis, surgical treatment, and oncologic outcome of this case. MDPI 2023-08-29 /pmc/articles/PMC10488709/ /pubmed/37685699 http://dx.doi.org/10.3390/jcm12175632 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Tan, Yi Liang
Ong, Wilson
Tan, Jiong Hao
Kumar, Naresh
Hallinan, James Thomas Patrick Decourcy
Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
title Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
title_full Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
title_fullStr Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
title_full_unstemmed Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
title_short Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report
title_sort epithelioid sarcoma of the spine: a review of literature and case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10488709/
https://www.ncbi.nlm.nih.gov/pubmed/37685699
http://dx.doi.org/10.3390/jcm12175632
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