Intracranial mesenchymal tumor, FET::CREB fusion-positive in the lateral ventricle.
Intracranial mesenchymal tumor, FET::CREB fusion-positive in the lateral ventricle.
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DOI：
10.1093/noajnl/vdad026
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发表时间：
2023-01
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颅内间叶肿瘤，FET：：CREB融合阳性，是2021年WHO中枢神经系统肿瘤分类中最近认可的临时实体。1这些是间叶性非脑膜皮肿瘤，具有广泛的形态谱。2-4有丝分裂活性通常较低（< 5个有丝分裂/mm 2）。建筑模式从合胞体或片状到网状索状结构。间质可以是胶原性或粘液性的。FET RNA结合蛋白家族基因（EWSR 1和FUS）与CREB家族转录因子（ATF 1、CREB和CREM）成员之间的融合是诊断性的。1具体而言，EWSR 1：：CREB融合已在血管瘤样纤维组织细胞瘤和透明细胞肉瘤中显示。5，6不再推荐该实体既往的疾病分类学名称“血管瘤样纤维组织细胞瘤颅内粘液样变异体"、”EWSR 1：：CREB家族基因融合的颅内粘液样间叶性肿瘤”。我们报告一例成人侧脑室的颅内间叶肿瘤，FET：：CREB融合阳性。
Intracranial mesenchymal tumor, FET:: CREB fusion-positive, is a recently recognized provisional entity in the 2021 WHO Classification of Tumors of the Central Nervous System. 1 These are mesenchymal, nonmeningothelial tumors, with a wide morphologic spectrum. 2–4 Mitotic activity is usually low (< 5 mitoses/mm2). Architectural patterns vary from syncytial or sheet-like to reticular cord-like structures. The stroma can be collagenous or myxoid. A fusion between a FET RNA-binding protein family gene (EWSR1 and FUS) and a member of the CREB family of transcription factors (ATF1, CREB, and CREM) is diagnostic. 1 Specifically, EWSR1:: CREB fusions have been shown in angiomatoid fibrous histiocytoma and clear cell sarcoma. 5, 6 Prior nosological designations “intracranial myxoid variant of angiomatoid fibrous histiocytoma,”“intracranial myxoid mesenchymal tumor with EWSR1:: CREB family gene fusions” are no longer recommended for this entity. 7, 8 We report a case of intracranial mesenchymal tumor, FET:: CREB fusion-positive, in lateral ventricle of an adult patient.