eISSN: 2084-9869
ISSN: 1233-9687
Polish Journal of Pathology
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4/2023
vol. 74
 
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abstract:
Case report

Biphenotypic sinonasal sarcoma with PAX3/FOXO1 fusion

Ilkay Tosun
1
,
Murat H. Karabulut
1
,
Ismail Yilmaz
2
,
Ahmet A. Cırık
3
,
Onur Sahin
1

1.
Department of Pathology, University of Health Sciences, Umraniye Training and Research Hospital, Istanbul, Turkey
2.
Department of Pathology, University of Health Sciences, Sultan 2. Abdul Hamid Khan Educational and Research Hospital, Istanbul, Turkey
3.
Department of Otolaryngology, University of Healt Sciences, Umraniye Training and Research Hospital, Istanbul, Turkey
Pol J Pathol 2023; 74 (4): 282-285
DOI: https://doi.org/10.5114/pjp.2023.134321
Online publish date: 2024/02/01
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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.
keywords:

biphenotypic sinonasal sarcoma, PAX3/FOXO1, low-grade neoplasia
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