消化系统混合性腺瘤伴分化好的神经内分泌肿瘤(MANET) 混合性神经内分泌-非神经内分泌肿瘤(MiNEN)的惰性亚型

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  混合性神经内分泌-非神经内分泌肿瘤(mixed neuroendocrine-nonneuroendocrine neoplasm, MiNEN)由腺瘤与分化好的神经内分泌肿瘤(neuroendocrine tumor, NET)组成,最近被定义为"MANET"。然而,这类肿瘤的临床病理特点及发病机制并不明确。我们通过检测12例MANET病例中p53和β-联蛋白的表达及瘤体中2种不同肿瘤成分的微卫星不稳定和KRAS、BRAF以及PIK3CA基因突变来分析这类肿瘤的临床病理特点。所有病例中,腺瘤成分都是病变的主体,而NET成分则位于息肉深部的中央。根据NET成分将肿瘤分级,9例为NET G1, 2例为NET G2, 1例为NET G3。所有病例中,腺体与NET成分混杂生长,二者之间可见移行区域。所有病例NET成分的p53均为阴性,8例中有3例出现不同程度的β-联蛋白细胞核阳性。所有病例平均随访9年后均无病存活。肿瘤中两种成分均未检测到KRAS、BRAF和PIK3CA基因突变以及微卫星不稳定。我们回顾之前文献报 道过的59例MANET,也未发现因为肿瘤导致的死亡。伴有低分化神经内分泌癌成分的高级别恶性MiNEN,类似于混合性腺瘤-神经内分泌癌,目前推测两种肿瘤中的成分可能是同一来源。因此,尽管目前研究表明MANET是一种惰性肿瘤,但仍需和侵袭性高级别MiNEN相鉴别。
Am J Surg Pathol 2018;42:1503-1512
美国外科病理学杂志中文版2019年第二期摘要NO.7
(许素素 翻译  滕梁红 审校)
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