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Myoepithelioma-like Hyalinizing Epithelioid Tumors of the Hand With Novel OGT-FOXO3 Fusions.

手部肌上皮瘤样透明样上皮样肿瘤伴新型OGT-FOXO3融合。

  • 影响因子:6.06
  • DOI:10.1097/PAS.0000000000001380
  • 作者列表:"Lee JC","Chou HC","Wang CH","Chu PY","Hsieh TH","Liu ML","Hsieh SM","Liu YR","Kao YC
  • 发表时间:2020-03-01
Abstract

:Myoepithelial tumors of soft tissue are uncommon neoplasms characterized histologically by spindle to epithelioid cells arranged in cords, nests, and/or reticular pattern with chondromyxoid to hyaline stroma, and genetically by rearrangement involving EWSR1 (among other less common genes) in about half of the cases. The diagnosis often requires immunostaining to confirm myoepithelial differentiation, most importantly the expression of epithelial markers and S100 protein and/or GFAP. However, there are cases wherein the morphology is reminiscent of myoepithelial tumors, while the immunophenotype falls short. Here, we report 2 highly similar myoepithelioma-like tumors arising in the hands of young adults. Both tumors were well-demarcated and composed of alternating cellular areas with palely eosinophilic hyaline stroma and scattered acellular zones of densely eosinophilic collagen deposition. The tumor cells were mainly epithelioid cells and arranged in cords or small nests. Vacuolated cells encircling hyaline matrix globules were focally prominent. A minor component of nonhyaline fibrous nodular areas composed of bland spindle cells and rich vasculature was also observed. Perivascular concentric spindle cell proliferation and perivascular hyalinization were present in some areas. The tumor cells were positive for CD34 and epithelial membrane antigen (focal) by immunostaining, while largely negative for cytokeratin, S100, GFAP, p63, GLUT1, and claudin-1. By RNA sequencing, a novel OGT-FOXO3 fusion gene was identified in case 1 and confirmed by reverse transcription polymerase chain reaction and fluorescence in situ hybridization in both cases. Sharing the unusual clinicopathologic features and the novel fusion, these 2 cases probably represent a distinct tumor entity, whose relationship with myoepithelial tumors and tumorigenic mechanisms exerted by the OGT-FOXO3 fusion remain to be studied.

摘要

: 软组织的肌上皮肿瘤在组织学上是不常见的肿瘤,其特征在于纺锤体至上皮样细胞排列成索、巢和/或网状图案,软骨粘液样至透明基质,并且在约一半的病例中遗传上涉及EWSR1 (在其他不太常见的基因中) 的重排。诊断通常需要免疫染色以确认肌上皮分化,最重要的是上皮标志物和S100蛋白和/或GFAP的表达。然而,有些情况下,形态令人联想到肌上皮肿瘤,而免疫表型不足。在这里,我们报告了2个非常相似的肌上皮瘤样肿瘤发生在年轻人手中。两种肿瘤边界清楚,由交替的细胞区域组成,这些区域具有苍白的嗜酸性透明基质和密集嗜酸性胶原沉积的散在脱细胞区。肿瘤细胞以上皮样细胞为主,呈索状或小巢状排列。空泡细胞环绕透明基质小球,局灶突出。还观察到由淡薄的梭形细胞和丰富的脉管系统组成的非透明纤维结节区的次要成分。部分区域存在血管周围同心梭形细胞增殖和血管周围玻璃样变。通过免疫染色,肿瘤细胞对CD34和上皮膜抗原 (病灶) 呈阳性,而对细胞角蛋白、S100、GFAP、p63、GLUT1和claudin-1大部分呈阴性。通过RNA测序,在病例1中鉴定了新的OGT-FOXO3融合基因,并在两种情况下通过逆转录聚合酶链反应和荧光原位杂交证实。这两个病例具有不同寻常的临床病理特征和新的融合,可能代表了一个不同的肿瘤实体,其与肌上皮肿瘤的关系以及由OGT-FOXO3融合产生的致瘤机制仍有待研究。

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软组织肿瘤方向

软组织肿瘤是起源于间叶组织位于软组织内的肿瘤。主要是运动系统的软组织(如肌肉、韧带、骨膜、脂肪等)肿瘤。良性者为瘤,恶性者为肉瘤。

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