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Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine

BACKGROUND: Epithelioid sarcoma is rare, represents less than 1% of all sarcomas, usually occurs in the extremities, and rarely presents as a primary sarcoma of the spine. Publications are usually single reports or case series. We aimed to undertake a systematic review of publications of cases of pr...

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Autores principales: Hu, Weiran, Wu, Xiaonan, Ma, Haohao, Wang, Hongqiang, Shi, Xinge, Zhang, Kai, Gao, Yanzheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scientific Literature, Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9830813/
https://www.ncbi.nlm.nih.gov/pubmed/36604864
http://dx.doi.org/10.12659/MSM.938416
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author Hu, Weiran
Wu, Xiaonan
Ma, Haohao
Wang, Hongqiang
Shi, Xinge
Zhang, Kai
Gao, Yanzheng
author_facet Hu, Weiran
Wu, Xiaonan
Ma, Haohao
Wang, Hongqiang
Shi, Xinge
Zhang, Kai
Gao, Yanzheng
author_sort Hu, Weiran
collection PubMed
description BACKGROUND: Epithelioid sarcoma is rare, represents less than 1% of all sarcomas, usually occurs in the extremities, and rarely presents as a primary sarcoma of the spine. Publications are usually single reports or case series. We aimed to undertake a systematic review of publications of cases of primary epithelioid sarcoma of the spine to evaluate clinical presentation, diagnosis, management, and patient outcomes. MATERIAL/METHODS: We searched studies on spinal epithelioid sarcoma in the PubMed database. Only studies with secondary epithelioid sarcoma or without effective data for analysis were excluded. Cases in which epithelioid sarcoma first invaded other sites and then affected the spine were also excluded. RESULTS: Twenty-three patients from 13 studies were included in the study, aged between 14 and 65 years, and the sex ratio of female to male was 1: 2.29. The survival time was 18.7±13.8 months. The survival time of males was longer than that of females (22.9±14.4 vs 9.0±4.6, P=0.027). The onset age was linearly correlated with the size of the lesion (size=−0.161*age+11.841).The lesions located in lumbar vertebra had the worst prognosis. Postoperative radiotherapy had a statistically significant effect on survival time (P=0.040). CONCLUSIONS: This systematic review identified 23 published cases of primary epithelioid sarcoma of the spine. Pain was the main presenting symptom, and tumor size increased with patient age. Female sex and primary location in the lumbar spine were associated with poor survival. Although surgery was the first-line treatment, postoperative radiotherapy and chemotherapy may improve clinical outcomes.
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spelling pubmed-98308132023-01-26 Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine Hu, Weiran Wu, Xiaonan Ma, Haohao Wang, Hongqiang Shi, Xinge Zhang, Kai Gao, Yanzheng Med Sci Monit Clinical Research BACKGROUND: Epithelioid sarcoma is rare, represents less than 1% of all sarcomas, usually occurs in the extremities, and rarely presents as a primary sarcoma of the spine. Publications are usually single reports or case series. We aimed to undertake a systematic review of publications of cases of primary epithelioid sarcoma of the spine to evaluate clinical presentation, diagnosis, management, and patient outcomes. MATERIAL/METHODS: We searched studies on spinal epithelioid sarcoma in the PubMed database. Only studies with secondary epithelioid sarcoma or without effective data for analysis were excluded. Cases in which epithelioid sarcoma first invaded other sites and then affected the spine were also excluded. RESULTS: Twenty-three patients from 13 studies were included in the study, aged between 14 and 65 years, and the sex ratio of female to male was 1: 2.29. The survival time was 18.7±13.8 months. The survival time of males was longer than that of females (22.9±14.4 vs 9.0±4.6, P=0.027). The onset age was linearly correlated with the size of the lesion (size=−0.161*age+11.841).The lesions located in lumbar vertebra had the worst prognosis. Postoperative radiotherapy had a statistically significant effect on survival time (P=0.040). CONCLUSIONS: This systematic review identified 23 published cases of primary epithelioid sarcoma of the spine. Pain was the main presenting symptom, and tumor size increased with patient age. Female sex and primary location in the lumbar spine were associated with poor survival. Although surgery was the first-line treatment, postoperative radiotherapy and chemotherapy may improve clinical outcomes. International Scientific Literature, Inc. 2023-01-06 /pmc/articles/PMC9830813/ /pubmed/36604864 http://dx.doi.org/10.12659/MSM.938416 Text en © Med Sci Monit, 2023 https://creativecommons.org/licenses/by-nc-nd/4.0/This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/) )
spellingShingle Clinical Research
Hu, Weiran
Wu, Xiaonan
Ma, Haohao
Wang, Hongqiang
Shi, Xinge
Zhang, Kai
Gao, Yanzheng
Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine
title Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine
title_full Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine
title_fullStr Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine
title_full_unstemmed Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine
title_short Systematic Review of Published Cases of Primary Epithelioid Sarcoma of the Spine
title_sort systematic review of published cases of primary epithelioid sarcoma of the spine
topic Clinical Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9830813/
https://www.ncbi.nlm.nih.gov/pubmed/36604864
http://dx.doi.org/10.12659/MSM.938416
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