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P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm

INTRODUCTION: The classification of soft tissue and bone neoplasms is experiencing a transformation as methods to detect gene fusions expand. Next-generation sequencing panels, anchored multiplex polymerase chain reaction systems and comprehensive RNA sequencing have all contributed to new histologi...

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Autores principales: Hardman, Lotte, Ibriham, Mina, Khundkar, Roba, Wilson, Paul
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8030262/
http://dx.doi.org/10.1093/bjsopen/zrab032.134
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author Hardman, Lotte
Ibriham, Mina
Khundkar, Roba
Wilson, Paul
author_facet Hardman, Lotte
Ibriham, Mina
Khundkar, Roba
Wilson, Paul
author_sort Hardman, Lotte
collection PubMed
description INTRODUCTION: The classification of soft tissue and bone neoplasms is experiencing a transformation as methods to detect gene fusions expand. Next-generation sequencing panels, anchored multiplex polymerase chain reaction systems and comprehensive RNA sequencing have all contributed to new histological categorisation. The EWSR1-NFATC2 is one such novel translocation found in a round cell sarcomas. These sarcoma sit within the WHO group of undifferentiated round and spindle cell sarcomas. They presents as either a primary bone or soft tissue tumour and exhibits distinctive histopathologic features. METHODS: This is a case study of a soft tissue mesenchymal tumour with the recently described gene fusion EWSR1-NFATC2. The case is described, with 3-month outcomes and considered in the most recent literature on this sarcoma subset (26 cases). RESULTS: This case study prompted a look at the current NHS paradigm for these emerging histopathologically distinctive sarcoma subgroups. At present the expertise with the sarcoma MDT, along with second opinion from other national MDTs is the format for subtype specific sarcoma management, informed by recent literature. CONCLUSION: The EWSR1-NFATC2 fusion round cell sarcoma tumours are an example of a very recently emerged sarcoma subset which display oncological variance to the wider Ewing’s family. The novel literature guided the Southmead and Royal Marsden MDT opinions to proceed straight to surgical excision. The Authors recommend a national prospective database for rare sarcoma subsets, particularly those that appear to exhibit distinctive clinicopathology characteristics. The format could be through the RSTN collaborative, secure anonymised data collection can be collected through REDCap.
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spelling pubmed-80302622021-04-13 P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm Hardman, Lotte Ibriham, Mina Khundkar, Roba Wilson, Paul BJS Open Poster Presentation INTRODUCTION: The classification of soft tissue and bone neoplasms is experiencing a transformation as methods to detect gene fusions expand. Next-generation sequencing panels, anchored multiplex polymerase chain reaction systems and comprehensive RNA sequencing have all contributed to new histological categorisation. The EWSR1-NFATC2 is one such novel translocation found in a round cell sarcomas. These sarcoma sit within the WHO group of undifferentiated round and spindle cell sarcomas. They presents as either a primary bone or soft tissue tumour and exhibits distinctive histopathologic features. METHODS: This is a case study of a soft tissue mesenchymal tumour with the recently described gene fusion EWSR1-NFATC2. The case is described, with 3-month outcomes and considered in the most recent literature on this sarcoma subset (26 cases). RESULTS: This case study prompted a look at the current NHS paradigm for these emerging histopathologically distinctive sarcoma subgroups. At present the expertise with the sarcoma MDT, along with second opinion from other national MDTs is the format for subtype specific sarcoma management, informed by recent literature. CONCLUSION: The EWSR1-NFATC2 fusion round cell sarcoma tumours are an example of a very recently emerged sarcoma subset which display oncological variance to the wider Ewing’s family. The novel literature guided the Southmead and Royal Marsden MDT opinions to proceed straight to surgical excision. The Authors recommend a national prospective database for rare sarcoma subsets, particularly those that appear to exhibit distinctive clinicopathology characteristics. The format could be through the RSTN collaborative, secure anonymised data collection can be collected through REDCap. Oxford University Press 2021-04-08 /pmc/articles/PMC8030262/ http://dx.doi.org/10.1093/bjsopen/zrab032.134 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of BJS Society Ltd. https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercialre-use, please contact journals.permissions@oup.com
spellingShingle Poster Presentation
Hardman, Lotte
Ibriham, Mina
Khundkar, Roba
Wilson, Paul
P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm
title P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm
title_full P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm
title_fullStr P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm
title_full_unstemmed P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm
title_short P135 Undifferentiated round cell and spindle cell sarcomas: the current paradigm
title_sort p135 undifferentiated round cell and spindle cell sarcomas: the current paradigm
topic Poster Presentation
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8030262/
http://dx.doi.org/10.1093/bjsopen/zrab032.134
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