Department of Diagnostic Pathology, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo, 650-0017, Japan.
Department of Diagnostic Pathology, National Cancer Center Hospital, 5-5-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Brain Tumor Pathol. 2020 Apr;37(2):76-80. doi: 10.1007/s10014-020-00359-x. Epub 2020 Mar 25.
Intracranial myxoid mesenchymal tumors harboring EWSR1 fusions with CREB transcriptional factor gene families were recently described in several case reports and a few case series and this tumor closely resembles the myxoid variant of angiomatoid fibrous histiocytoma. We herein present an intracranial mesenchymal myxoid tumor arising in the third ventricle of a middle-aged woman. The tumor displayed prominent myxoid features consisting of mildly atypical oval to round cells, arranged in reticular and cord-like structures, with starburst-like amianthoid fibers, whereas it lacked pseudoangiomatoid spaces, pseudocapsules and lymphoid cuffing. Immunophenotypically, tumor cells were positive for EMA, desmin, and ALK (focal). EWSR1 and CREB1 rearrangements were identified using FISH assay. The proliferation index was low. It is currently uncertain whether these myxoid tumors represent a variant of angiomatoid fibrous histiocytoma or a novel tumor entity.
颅内黏液样间叶性肿瘤，具有 EWSR1 融合与 CREB 转录因子基因家族，最近在几例病例报告和一些病例系列中被描述，这种肿瘤与血管外皮瘤样纤维组织细胞瘤的黏液样变体密切相似。我们在此介绍一例发生在中年女性第三脑室的颅内间叶性黏液样肿瘤。肿瘤表现出明显的黏液样特征，由轻度非典型的椭圆形至圆形细胞组成，呈网状和索状结构，具有星爆状的石棉样纤维，而缺乏假血管瘤样空间、假包膜和淋巴套袖。免疫表型上，肿瘤细胞阳性表达 EMA、结蛋白和 ALK（局灶性）。使用 FISH 检测鉴定 EWSR1 和 CREB1 重排。增殖指数低。目前尚不确定这些黏液样肿瘤是否代表血管外皮瘤样纤维组织细胞瘤的变体或新的肿瘤实体。
