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.

中文翻译：

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扩展 EWSR1::CREM 融合肿瘤的谱系：一种罕见的儿科鼻内粘液样肿瘤。

EWSR1::CREM 基因融合在多种软组织肿瘤中得到越来越多的认识，包括明确定义的实体，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，EWSR1::CREM 融合肿瘤通常表现出原始梭形细胞或上皮样细胞、粘液样基质和广泛的免疫表型。在此，我们介绍了一名被诊断患有鼻内恶性粘液样肿瘤的儿童的不寻常病例，该肿瘤含有 EWSR1::CREM 基因融合体。据我们所知，这是第一例具有这种特殊融合的鼻内粘液样肿瘤。讨论了该病例的诊断和处理。