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Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature

BACKGROUND: Gliosarcoma is a rare variant of IDH- wild type glioblastoma with both glial and mesenchymal differentiation. It accounts for approximately 2% of glioblastomas and has a poor prognosis similar to that of classic glioblastoma. It is seen mostly between 40 and 60 years of age with a mean a...

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Autores principales: Din, Nasir Ud, Ishtiaq, Hira, Rahim, Shabina, Abdul-Ghafar, Jamshid, Ahmad, Zubair
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7908689/
https://www.ncbi.nlm.nih.gov/pubmed/33637068
http://dx.doi.org/10.1186/s12887-021-02556-9
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author Din, Nasir Ud
Ishtiaq, Hira
Rahim, Shabina
Abdul-Ghafar, Jamshid
Ahmad, Zubair
author_facet Din, Nasir Ud
Ishtiaq, Hira
Rahim, Shabina
Abdul-Ghafar, Jamshid
Ahmad, Zubair
author_sort Din, Nasir Ud
collection PubMed
description BACKGROUND: Gliosarcoma is a rare variant of IDH- wild type glioblastoma with both glial and mesenchymal differentiation. It accounts for approximately 2% of glioblastomas and has a poor prognosis similar to that of classic glioblastoma. It is seen mostly between 40 and 60 years of age with a mean age over 50 years. Pediatric gliosarcoma is even rarer than gliosarcoma in adults. We describe the clinicopathological features of gliosarcoma in patients under 20 years of age and determine whether there are significant differences from gliosarcoma in adults. We also present detailed review of published literature on pediatric gliosarcoma. METHODS: Slides of gliosarcomas in patients under 20 years of age were reviewed. Clinicopathological features were noted in detail and follow up was obtained. RESULTS: Eleven cases of gliosarcoma were reported in patients under 20 years of age. Ages ranged from three to 19 years (mean age 13 years). Frontal, parietal and temporal lobes were the commonest locations. Mean and median tumor size was six and five cm respectively. All 11 cases demonstrated the classic biphasic pattern. In 10 cases, glial component was astrocytic and was highlighted on GFAP. Sarcomatous component in most cases resembled fibrosarcoma and was high grade in 72.7%. Glial areas were reticulin poor while sarcomatous areas were reticulin rich. In over 45% cases, bizarre tumor giant cells were seen in the sarcomatous areas. In 1 case, sarcomatous areas showed extensive bone and cartilage formation. Other histologic features included hyalinized blood vessels, hemorrhage, infarction, gemistocytic cells, rhabdoid cells etc. Follow up was available in nine patients, five received chemoradiation post resection while three received radiotherapy only. Prognosis was dismal and eight patients died within one to 14 months following resection. CONCLUSIONS: Gliosarcomas in patients under 20 comprised 13% of all gliosarcomas reported during the study period. Frequency and mean age were higher compared to other published reports. Pathological features were similar to those described in literature. Clinicopathological features and prognosis of pediatric gliosarcomas were similar to adult gliosarcomas.
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spelling pubmed-79086892021-02-26 Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature Din, Nasir Ud Ishtiaq, Hira Rahim, Shabina Abdul-Ghafar, Jamshid Ahmad, Zubair BMC Pediatr Research Article BACKGROUND: Gliosarcoma is a rare variant of IDH- wild type glioblastoma with both glial and mesenchymal differentiation. It accounts for approximately 2% of glioblastomas and has a poor prognosis similar to that of classic glioblastoma. It is seen mostly between 40 and 60 years of age with a mean age over 50 years. Pediatric gliosarcoma is even rarer than gliosarcoma in adults. We describe the clinicopathological features of gliosarcoma in patients under 20 years of age and determine whether there are significant differences from gliosarcoma in adults. We also present detailed review of published literature on pediatric gliosarcoma. METHODS: Slides of gliosarcomas in patients under 20 years of age were reviewed. Clinicopathological features were noted in detail and follow up was obtained. RESULTS: Eleven cases of gliosarcoma were reported in patients under 20 years of age. Ages ranged from three to 19 years (mean age 13 years). Frontal, parietal and temporal lobes were the commonest locations. Mean and median tumor size was six and five cm respectively. All 11 cases demonstrated the classic biphasic pattern. In 10 cases, glial component was astrocytic and was highlighted on GFAP. Sarcomatous component in most cases resembled fibrosarcoma and was high grade in 72.7%. Glial areas were reticulin poor while sarcomatous areas were reticulin rich. In over 45% cases, bizarre tumor giant cells were seen in the sarcomatous areas. In 1 case, sarcomatous areas showed extensive bone and cartilage formation. Other histologic features included hyalinized blood vessels, hemorrhage, infarction, gemistocytic cells, rhabdoid cells etc. Follow up was available in nine patients, five received chemoradiation post resection while three received radiotherapy only. Prognosis was dismal and eight patients died within one to 14 months following resection. CONCLUSIONS: Gliosarcomas in patients under 20 comprised 13% of all gliosarcomas reported during the study period. Frequency and mean age were higher compared to other published reports. Pathological features were similar to those described in literature. Clinicopathological features and prognosis of pediatric gliosarcomas were similar to adult gliosarcomas. BioMed Central 2021-02-26 /pmc/articles/PMC7908689/ /pubmed/33637068 http://dx.doi.org/10.1186/s12887-021-02556-9 Text en © The Author(s) 2021 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research Article
Din, Nasir Ud
Ishtiaq, Hira
Rahim, Shabina
Abdul-Ghafar, Jamshid
Ahmad, Zubair
Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature
title Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature
title_full Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature
title_fullStr Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature
title_full_unstemmed Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature
title_short Gliosarcoma in patients under 20 years of age. A clinicopathologic study of 11 cases and detailed review of the literature
title_sort gliosarcoma in patients under 20 years of age. a clinicopathologic study of 11 cases and detailed review of the literature
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7908689/
https://www.ncbi.nlm.nih.gov/pubmed/33637068
http://dx.doi.org/10.1186/s12887-021-02556-9
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