Kervarrec Thibault, Lecointre Claire, Kerdraon Rémy, Bens Guido, Piquard Arnaud, Michenet Patrick
Service d'anatomie pathologique, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France.
Service d'anatomie pathologique, hôpital de la Source, 14, avenue de l'Hôpital, 45067 Orléans, France.
Ann Pathol. 2015 Dec;35(6):506-10. doi: 10.1016/j.annpat.2015.09.006. Epub 2015 Nov 14.
The gastro-intestinal neuroectodermal tumor (GNET) is a rare sarcoma of the digestive tract, which was recently recognised. The knowledge of the morphological, immunohistochemical and molecular diagnostic criteria is necessary to not mistake it for the metastasis of a melanoma or for another sarcoma of the digestive tract as the gastro-intestinal clear cells sarcoma or the malignant peripheral nervous system tumor (MPNST). We report the case of a 41-year-old patient with a GNET of the small intestine with hepatic metastasis. The histological examination showed a diffuse proliferation of epithelioid cells, which only express PS100. The presence EWSR1-ATF1 gene fusions with any melanocytic differentiation leads to the diagnosis of GNET.
胃肠道神经外胚层肿瘤（GNET）是一种罕见的消化道肉瘤，最近才被认识。了解其形态学、免疫组织化学和分子诊断标准对于不将其误诊为黑色素瘤转移或其他消化道肉瘤（如胃肠道透明细胞肉瘤或恶性外周神经鞘膜瘤（MPNST））很有必要。我们报告了一例41岁小肠GNET伴肝转移的患者。组织学检查显示上皮样细胞弥漫性增生，仅表达PS100。存在EWSR1-ATF1基因融合且无任何黑素细胞分化可诊断为GNET。
