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2020年WHO分类软组织肿瘤病理学新进展(2)
EWSR1-SMAD3-positive Fibroblastic Tumor | EWSR1-SMAD3阳性纤维母细胞肿瘤 |
This provisional entity is a benign fibroblastic neoplasm, with recent reports describing a wide age range, female predilection, and distribution largely in the hands and feet, in superficial (dermal or subcutaneous) locations. | 这个暂定实体是一种良性纤维母细胞肿瘤,最近报告描述的年龄范围广,女性多见,主要分布在手和足的浅表(真皮或皮下)部位。 |
Histologic features include nodular growth, a pattern of zonation demonstrating peripheral areas of intersecting cellular fascicles comprised of bland spindle cells (Fig. 4), and sometimes central areas of hyalinization and infarction. | 组织学特征包括结节状生长,显示由温和的梭形细胞组成的交叉细胞束的周围区域呈带状分布(图4),有时中心区域出现玻璃样变和梗死。 |
IHC reveals diffuse ERG expression, whereas SMA and CD34 are typically negative. This tumor type is defined by fusion of exon 7 of EWSR1 with exon 5 of SMAD3; SMAD3 is an important signal transducer in the TGF-β/SMAD pathway, involved in extracellular matrix synthesis by fibroblasts. This benign neoplasm may recur locally following incomplete excision. | IHC显示弥漫性ERG表达,而SMA和CD34通常为阴性。这种肿瘤类型由EWSR1的第7外显子和SMAD3的第5外显子融合而成;SMAD3是TGF-β/SMAD通路中的一个重要信号转导子,参与成纤维细胞合成细胞外基质。这种良性肿瘤在不完全切除后可能局部复发。 |
FIGURE 4. EWSR1-SMAD3-positive fibroblastic tumor. A, Intersecting fascicles of spindle cells show irregular margins with adjacent subcutaneous adipose tissue. B, The fibroblastic spindle cells contain uniform nuclei and pale eosinophilic cytoplasm.
图4 EWSR1-SMAD3阳性纤维母细胞瘤:A、梭形细胞的交叉束显示与邻近皮下脂肪组织不规则的边缘。B、纤维母细胞梭形细胞细胞核一致,胞质淡嗜酸性。
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