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拓展融合肿瘤谱:一种罕见的小儿鼻腔黏液样肿瘤。

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

机构信息

Department of Otolaryngology, KK Women's and Children's Hospital, Singapore, Singapore.

Department of Pathology and Laboratory Medicine, KK Women's and Children's Hospital, Singapore, Singapore.

出版信息

Pediatr Dev Pathol. 2024 Jan-Feb;27(1):90-95. doi: 10.1177/10935266231199931. Epub 2023 Oct 11.

DOI:10.1177/10935266231199931
PMID:37818644
Abstract

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, 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 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.

摘要

基因融合在多种软组织肿瘤中越来越被认识,包括明确的实体,如血管瘤样纤维组织细胞瘤或透明细胞肉瘤,以及其他无法分类的肿瘤。作为一个群体,融合肿瘤通常表现为原始梭形或上皮样细胞、黏液样基质和广泛的免疫表型。本文介绍了一例儿童鼻腔内恶性黏液样肿瘤的罕见病例,该肿瘤存在 基因融合。据我们所知,这是首例具有这种特殊融合的鼻腔黏液样肿瘤。讨论了该病例的诊断和治疗。

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