伴有 PAX3/FOXO1 融合的双型鼻窦肉瘤

IF 0.7 4区医学 Q4 PATHOLOGY

Polish Journal of Pathology Pub Date : 2023-01-01 DOI:10.5114/pjp.2023.134321

Ilkay Tosun, Murat H Karabulut, Ismail Yilmaz, Ahmet A Cırık, Onur Sahin

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引用次数: 0

摘要

双型鼻窦肉瘤（BSNS）以前被称为低分化鼻窦肉瘤，是一种罕见的鼻窦肿瘤，于2012年首次被描述。它涉及肌源性和神经性分化，以 PAX3 重排为特征。MAML3是PAX3最常见的融合伙伴；但在一部分病例中，其伙伴仍未确定。这些肿瘤的局部复发率很高，但缺乏转移潜力。在此，我们报告了一例伴有 PAX3/FOXO1 融合的 BSNS，并讨论了其临床病理特征和鉴别诊断。

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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; 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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来源期刊

Polish Journal of Pathology PATHOLOGY-

CiteScore

1.00

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： Polish Journal of Pathology is an official magazine of the Polish Association of Pathologists and the Polish Branch of the International Academy of Pathology. For the last 18 years of its presence on the market it has published more than 360 original papers and scientific reports, often quoted in reviewed foreign magazines. A new extended Scientific Board of the quarterly magazine comprises people with recognised achievements in pathomorphology and biology, including molecular biology and cytogenetics, as well as clinical oncology. Polish scientists who are working abroad and are international authorities have also been invited. Apart from presenting scientific reports, the magazine will also play a didactic and training role.