Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion

Biphenotypic sinonasal sarcoma (BSNS), previously known as low-grade sinonasal sarcoma, is a rare tumour of the sinonasal tract, first described in 2012. It involves both myogenic and neural differentiation and is characterized by PAX3 rearrangement. MAML3 is the most frequent fusion partner of PAX3...

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Main Authors: Ilkay Tosun, Murat H. Karabulut, Ismail Yilmaz, Ahmet A. Cırık, Onur Sahin
Format: Article
Language:English
Published: Termedia Publishing House 2024-02-01
Series:Polish Journal of Pathology
Subjects:
Online Access:https://www.termedia.pl/Biphenotypic-sinonasal-sarcoma-with-PAX3-FOXO1-fusion,55,52242,1,1.html
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author Ilkay Tosun
Murat H. Karabulut
Ismail Yilmaz
Ahmet A. Cırık
Onur Sahin
author_facet Ilkay Tosun
Murat H. Karabulut
Ismail Yilmaz
Ahmet A. Cırık
Onur Sahin
author_sort Ilkay Tosun
collection DOAJ
description Biphenotypic sinonasal sarcoma (BSNS), previously known as low-grade sinonasal sarcoma, is a rare tumour of the sinonasal tract, first described in 2012. It involves both myogenic and neural differentiation and is characterized by PAX3 rearrangement. MAML3 is the most frequent fusion partner of PAX3; however, its partner remains unidentified in a subset of cases. These tumours have significant local recurrence rates but lack metastatic potential. Here, we report a case of BSNS with PAX3/FOXO1 fusion and discuss its clinicopathological features and differential diagnosis.
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spelling doaj.art-02972becac074f7eb9845af2bbcf92b52024-02-02T12:10:24ZengTermedia Publishing HousePolish Journal of Pathology1233-96872084-98692024-02-0174428228510.5114/pjp.2023.13432152242Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusionIlkay TosunMurat H. KarabulutIsmail YilmazAhmet A. CırıkOnur SahinBiphenotypic sinonasal sarcoma (BSNS), previously known as low-grade sinonasal sarcoma, is a rare tumour of the sinonasal tract, first described in 2012. It involves both myogenic and neural differentiation and is characterized by PAX3 rearrangement. MAML3 is the most frequent fusion partner of PAX3; however, its partner remains unidentified in a subset of cases. These tumours have significant local recurrence rates but lack metastatic potential. Here, we report a case of BSNS with PAX3/FOXO1 fusion and discuss its clinicopathological features and differential diagnosis.https://www.termedia.pl/Biphenotypic-sinonasal-sarcoma-with-PAX3-FOXO1-fusion,55,52242,1,1.htmlbiphenotypic sinonasal sarcoma pax3/foxo1 low-grade neoplasia.
spellingShingle Ilkay Tosun
Murat H. Karabulut
Ismail Yilmaz
Ahmet A. Cırık
Onur Sahin
Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion
Polish Journal of Pathology
biphenotypic sinonasal sarcoma
pax3/foxo1
low-grade neoplasia.
title Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion
title_full Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion
title_fullStr Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion
title_full_unstemmed Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion
title_short Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion
title_sort biphenotypic sinonasal sarcoma with pax3 foxo1 fusion
topic biphenotypic sinonasal sarcoma
pax3/foxo1
low-grade neoplasia.
url https://www.termedia.pl/Biphenotypic-sinonasal-sarcoma-with-PAX3-FOXO1-fusion,55,52242,1,1.html
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AT ismailyilmaz biphenotypicsinonasalsarcomawithpax3foxo1fusion
AT ahmetacırık biphenotypicsinonasalsarcomawithpax3foxo1fusion
AT onursahin biphenotypicsinonasalsarcomawithpax3foxo1fusion