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Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review

OBJECTIVE: Primary spinal anaplastic ependymoma (PSAE) is an extremely rare disease. We aim to report the largest PSAE cohort, evaluate the treatments, and investigate the prognostic factors for progression-free survival (PFS). METHODS: Clinical data collected from the authors’ institute and literat...

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Autores principales: Wu, Liang, Wang, Li’ao, Zou, Wanjing, Yang, Jun, Jia, Wenqing, Xu, Yulun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9941548/
https://www.ncbi.nlm.nih.gov/pubmed/36824145
http://dx.doi.org/10.3389/fonc.2023.1083085
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author Wu, Liang
Wang, Li’ao
Zou, Wanjing
Yang, Jun
Jia, Wenqing
Xu, Yulun
author_facet Wu, Liang
Wang, Li’ao
Zou, Wanjing
Yang, Jun
Jia, Wenqing
Xu, Yulun
author_sort Wu, Liang
collection PubMed
description OBJECTIVE: Primary spinal anaplastic ependymoma (PSAE) is an extremely rare disease. We aim to report the largest PSAE cohort, evaluate the treatments, and investigate the prognostic factors for progression-free survival (PFS). METHODS: Clinical data collected from the authors’ institute and literature articles were pooled and described. Survival analysis and multivariable Cox regression analysis were performed to evaluate therapies and investigate prognostic factors for PFS. RESULTS: Our cohort included 22 females and 16 males, with a median age of 33 years. PSAE developed mostly on cervical and cervicothoracic levels. The median length measured 3 segments. Half of PSAE were intramedullary. Pain was the most common symptom. The median duration of symptoms was 6 months. Neurological statuses were improved in 76% following treatments, whereas clinical tumor progression occurred in 41.7%. The estimated median progression-free survival was 132 months, and the estimated median survival was 192 months. The median Ki-67 index was 15%. Patients aged less than or equal to 25 experienced worse neurological statuses and more repeated progression. Age less than or equal to 25 (HR 10.312, 95%CI 1.535-69.260, p=0.016), gross total resection (HR 0.116, 95%CI 0.020-0.688, p=0.018), and radiotherapy (HR 0.084, 95%CI 0.009-0.804, p=0.032) are three prognostic factors for tumor progression. CONCLUSION: Tumor progression remains a big concern in the clinical course of PSAE. Being aged above 25, undergoing GTR, and accepting adjuvant radiotherapy put patients at lower risk for tumor progression. Younger patients might have worse neurological statuses compared with those aged over 25.
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spelling pubmed-99415482023-02-22 Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review Wu, Liang Wang, Li’ao Zou, Wanjing Yang, Jun Jia, Wenqing Xu, Yulun Front Oncol Oncology OBJECTIVE: Primary spinal anaplastic ependymoma (PSAE) is an extremely rare disease. We aim to report the largest PSAE cohort, evaluate the treatments, and investigate the prognostic factors for progression-free survival (PFS). METHODS: Clinical data collected from the authors’ institute and literature articles were pooled and described. Survival analysis and multivariable Cox regression analysis were performed to evaluate therapies and investigate prognostic factors for PFS. RESULTS: Our cohort included 22 females and 16 males, with a median age of 33 years. PSAE developed mostly on cervical and cervicothoracic levels. The median length measured 3 segments. Half of PSAE were intramedullary. Pain was the most common symptom. The median duration of symptoms was 6 months. Neurological statuses were improved in 76% following treatments, whereas clinical tumor progression occurred in 41.7%. The estimated median progression-free survival was 132 months, and the estimated median survival was 192 months. The median Ki-67 index was 15%. Patients aged less than or equal to 25 experienced worse neurological statuses and more repeated progression. Age less than or equal to 25 (HR 10.312, 95%CI 1.535-69.260, p=0.016), gross total resection (HR 0.116, 95%CI 0.020-0.688, p=0.018), and radiotherapy (HR 0.084, 95%CI 0.009-0.804, p=0.032) are three prognostic factors for tumor progression. CONCLUSION: Tumor progression remains a big concern in the clinical course of PSAE. Being aged above 25, undergoing GTR, and accepting adjuvant radiotherapy put patients at lower risk for tumor progression. Younger patients might have worse neurological statuses compared with those aged over 25. Frontiers Media S.A. 2023-02-07 /pmc/articles/PMC9941548/ /pubmed/36824145 http://dx.doi.org/10.3389/fonc.2023.1083085 Text en Copyright © 2023 Wu, Wang, Zou, Yang, Jia and Xu 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, Liang
Wang, Li’ao
Zou, Wanjing
Yang, Jun
Jia, Wenqing
Xu, Yulun
Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review
title Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review
title_full Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review
title_fullStr Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review
title_full_unstemmed Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review
title_short Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review
title_sort primary spinal anaplastic ependymoma: a single-institute retrospective cohort and systematic review
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9941548/
https://www.ncbi.nlm.nih.gov/pubmed/36824145
http://dx.doi.org/10.3389/fonc.2023.1083085
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