Abstract：
Malignant gastrointestinal neuroectodermal tumor (M-GNET) and clear cell sarcoma (CCS) of soft tissue represent closely related, extremely rare, malignant mesenchymal neoplasm of uncertain differentiation. Both entities are characterized genetically by the same molecular alterations represented by the presence of EWSR1-ATF1 and, more rarely, EWSR1-CREB1 fusion genes. The latter translocation seems to be more represented in M-GNET that, despite significant morphologic overlap with CCS, tends to lack overt features of melanocytic differentiation. Most M-GNET occur in the lower gastrointestinal tract, whereas occurrence in the upper tract has been reported only exceptionally. The differential diagnosis represents a major challenge, and accurate diagnosis impact significantly on therapeutic planning. We herein report the clinicopathologic features of a molecularly confirmed M-GNET that arose at the base of the tongue and review the pertinent literature.
摘要：
恶性胃肠神经外胚层瘤(M-GNET)与软组织透明细胞肉瘤(CCS)密切相关,极为罕见,分化不确定的恶性间充质肿瘤。这两个实体的遗传特征是EWSR1-ATF1的存在所代表的相同分子改变，很少,EWSR1-CREB1融合基因。后者的易位似乎在M-GNET中表现得更多，尽管与CCS有明显的形态学重叠，往往缺乏黑素细胞分化的明显特征。大多数M-GNET发生在下胃肠道，而发生在上束仅有例外报道。鉴别诊断是一个重大挑战，准确的诊断对治疗计划有显著影响。我们在此报告了在舌根产生的分子证实的M-GNET的临床病理特征，并回顾了相关文献。
