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Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma

Ewing's sarcoma is a rare and aggressive tumor that typically arises in the long bones of the extremities. It belongs in the family of small round blue cell tumors and is characterized immunohistochemically by diffuse CD99 expression and molecularly by one of several oncogenic translocations, m...

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Autores principales: Zhou, Maggie, Ko, Yen Chen Kevin, Charville, Gregory W., Ganjoo, Kristen N.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7772862/
https://www.ncbi.nlm.nih.gov/pubmed/33442361
http://dx.doi.org/10.1159/000511415
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author Zhou, Maggie
Ko, Yen Chen Kevin
Charville, Gregory W.
Ganjoo, Kristen N.
author_facet Zhou, Maggie
Ko, Yen Chen Kevin
Charville, Gregory W.
Ganjoo, Kristen N.
author_sort Zhou, Maggie
collection PubMed
description Ewing's sarcoma is a rare and aggressive tumor that typically arises in the long bones of the extremities. It belongs in the family of small round blue cell tumors and is characterized immunohistochemically by diffuse CD99 expression and molecularly by one of several oncogenic translocations, most commonly t(11;22)(q24;q12) between the EWSR1 gene and the FLI1 gene. Here we present a rare case of Ewing's sarcoma in the sinonasal tract with FUS-ERG gene arrangement that was regarded for almost a decade as a sinonasal-type hemangiopericytoma (glomangiopericytoma). This case illustrates the surprisingly prolonged natural history of Ewing's sarcoma that did not receive therapy for many years and the importance of considering alternative genetic translocations. Our experience suggests that the presence of diffuse CD99 membranous staining pattern in a small blue round cell tumor with morphology typical for Ewing's sarcoma but FISH negative for EWSR1 rearrangement should prompt consideration of FUS-ERG fusion.
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spelling pubmed-77728622021-01-12 Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma Zhou, Maggie Ko, Yen Chen Kevin Charville, Gregory W. Ganjoo, Kristen N. Case Rep Oncol Case Report Ewing's sarcoma is a rare and aggressive tumor that typically arises in the long bones of the extremities. It belongs in the family of small round blue cell tumors and is characterized immunohistochemically by diffuse CD99 expression and molecularly by one of several oncogenic translocations, most commonly t(11;22)(q24;q12) between the EWSR1 gene and the FLI1 gene. Here we present a rare case of Ewing's sarcoma in the sinonasal tract with FUS-ERG gene arrangement that was regarded for almost a decade as a sinonasal-type hemangiopericytoma (glomangiopericytoma). This case illustrates the surprisingly prolonged natural history of Ewing's sarcoma that did not receive therapy for many years and the importance of considering alternative genetic translocations. Our experience suggests that the presence of diffuse CD99 membranous staining pattern in a small blue round cell tumor with morphology typical for Ewing's sarcoma but FISH negative for EWSR1 rearrangement should prompt consideration of FUS-ERG fusion. S. Karger AG 2020-11-30 /pmc/articles/PMC7772862/ /pubmed/33442361 http://dx.doi.org/10.1159/000511415 Text en Copyright © 2020 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Zhou, Maggie
Ko, Yen Chen Kevin
Charville, Gregory W.
Ganjoo, Kristen N.
Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma
title Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma
title_full Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma
title_fullStr Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma
title_full_unstemmed Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma
title_short Sinonasal FUS-ERG-Rearranged Ewing's Sarcoma Mimicking Glomangiopericytoma
title_sort sinonasal fus-erg-rearranged ewing's sarcoma mimicking glomangiopericytoma
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7772862/
https://www.ncbi.nlm.nih.gov/pubmed/33442361
http://dx.doi.org/10.1159/000511415
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