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2020年WHO分类软组织肿瘤病理学新进展(2)

2020-10-05 14:40  阅读(491)  评论(0)  分类:软组织

EWSR1-SMAD3-positive Fibroblastic Tumor

EWSR1-SMAD3阳性纤维细胞肿瘤

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。


2020年WHO分类软组织肿瘤病理学新进展(2)

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.

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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