Expanding the Spectrum of EWSR1::CREM Fusion Tumors: An Unusual Pediatric Intranasal Myxoid Tumor

被引：0

作者：

Koh, Shamen ^{[1
]}

Punjabi, Lavisha S. ^{[2
]}

Chang, Kenneth Tou En ^{[2
]}

Wei Yang Teo, Neville ^{[3
]}

Ee Hoon Teo, Constance ^{[3
]}

Soh, Shui Yen ^{[4
]}

Kun Kiaang Tan, Henry ^{[1
,5
]}

机构：

[1] KK Womens & Childrens Hosp, Dept Otolaryngol, Singapore, Singapore

[2] KK Womens & Childrens Hosp, Dept Pathol & Lab Med, Singapore, Singapore

[3] Singapore Gen Hosp, Dept Otorhinolaryngol Head & Neck Surg, Singapore, Singapore

[4] KK Womens & Childrens Hosp, Dept Hematol Oncol, Singapore, Singapore

[5] KK Women & Childrens Hosp, Dept Otolaryngol, Singapore 229899, Singapore

来源：

PEDIATRIC AND DEVELOPMENTAL PATHOLOGY | 2024年 / 27卷 / 01期

关键词：

EWSR1; EWSR1::CREM; CREM; pediatric tumor; pediatric otolaryngology; intranasal myxoid tumor; CLEAR-CELL SARCOMA; MESENCHYMAL TUMOR; EWSR1-CREB1; VARIANT; CANCER;

D O I：

10.1177/10935266231199931

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

EWSR1::CREM gene fusions are increasingly being recognized in a diverse number of soft tissue tumors, including well-defined entities such as angiomatoid fibrous histiocytoma or clear cell sarcoma, and other unclassifiable tumors. As a group, EWSR1::CREM fused tumors often demonstrate primitive spindle or epithelioid cells, myxoid stroma, and a broad immunophenotype. Herein we present an unusual case of a child diagnosed with an intranasal malignant myxoid tumor harboring an EWSR1::CREM gene fusion. To the best of our knowledge, this is the first case of intranasal myxoid tumor with this particular fusion. Diagnosis and management of the case is discussed.

引用

页码：90 / 95

页数：6