Abstract:
Amphicrine neoplasms (ANs) are poorly understood epithelial malignancies composed of cells with co-existing exocrine-neuroendocrine features. Here, we report a recurrent mucin-producing gastric amphicrine tumor co-expressing neuroendocrine (chromogranin-A, synaptophysin, and CD56) and pancreatic acinar cell (BCL10 and trypsin) markers, arisen in a 64-year-old woman during adjuvant immunotherapy for melanoma. Ki-67 was < 2%. The gastric background context was atrophic gastritis. Next-generation sequencing showed MEN1 mutation (p.P71fs*42) coupled with loss of heterozygosity. The key lessons were as follows: (1) gastric ANs can show the co-existence of exocrine mucin-producing elements with neuroendocrine and pancreatic acinar differentiation; (2) they may represent a new entity arising in the context of atrophic gastritis and during immunotherapy; (3) they should be considered in the diagnostic workup of gastric neuroendocrine tumors; and (4) their molecular profile can show striking similarities with well-differentiated neuroendocrine tumors. These findings may be of help to improve the knowledge and the biological taxonomy of ANs.
摘要:
苯丙胺肿瘤(ANs)是由具有共存的外分泌神经内分泌特征的细胞组成的上皮恶性肿瘤。这里,我们报告了一种复发性的产生粘蛋白的胃苯丙胺肿瘤,共表达神经内分泌(嗜铬粒蛋白-A,突触素,和CD56)和胰腺腺泡细胞(BCL10和胰蛋白酶)标记,一名64岁女性在黑色素瘤辅助免疫疗法期间出现。Ki-67<2%。胃背景背景为萎缩性胃炎。下一代测序显示MEN1突变(p。P71fs*42)与杂合性丧失耦合。主要教训如下:(1)胃AN可以显示外分泌粘蛋白产生元件与神经内分泌和胰腺腺泡分化共存;(2)它们可能代表萎缩性胃炎和免疫治疗过程中出现的新实体;(3)在胃神经内分泌肿瘤的诊断检查中应考虑它们;(4)它们的分子谱可以显示出与分化良好的神经内分泌肿瘤惊人的相似性。这些发现可能有助于提高对ANs的认识和生物学分类学。