Abstract：
Sinonasal neuroendocrine neoplasms (SN-NENs) are rare and mostly include neuroendocrine carcinoma (NEC), whereas neuroendocrine tumor (NET) is exceptional in this site. Olfactory neuroblastoma (ONB) is a malignant neuroectodermal neoplasm arising in the nasal cavity. Albeit crucial for correct patients\' management, the distinction of high grade ONB from NEC is challenging and requires additional diagnostic markers. The transcription factor SATB2 has been recently introduced in routine diagnostics as an immunohistochemical marker of distal intestine differentiation. No specific data are available about SATB2 and GATA3 expression in SN-NENs. GATA3, SATB2, and, for comparison, CDX2 expression were investigated in a series of epithelial and non-epithelial SN-NENs. We collected 26 cases of ONB and 7 cases of epithelial SN-NENs diagnosed and treated in our Institution. ONBs were graded according to Hyams\' system and epithelial NENs were reclassified into 5 NECs, 1 MiNEN, and 1 amphicrine carcinoma. Immunohistochemistry was performed using standard automated protocols. Hyams\' grades 1-3 ONBs stained diffusely and intensely for SATB2, whereas grade 4 ONBs and NECs were globally negative. The non-neuroendocrine component of MiNEN and the amphicrine carcinoma were strongly positive. GATA3 was heterogeneously and unpredictably expressed in Hyams\' grades 1-3 ONBs, whereas grade 4 ONBs and NECs were completely negative. CDX2 was negative in all cases. Our study identifies, for the first time, SATB2 and GATA3 expression as features of Hyams\' grades 1-3 ONBs, expands the spectrum of SATB2 and GATA3-positive neoplasms, and suggests that Hyams\' grade 4 ONBs are not only clinically but also biologically different from low graded ONBs.
摘要：
鼻窦神经内分泌肿瘤（SN-NENs）少见，主要包括神经内分泌癌（NEC），而神经内分泌肿瘤（NET）在这个部位是例外的。嗅觉神经母细胞瘤（ONB）是一种在鼻腔中产生的恶性神经外胚层肿瘤。尽管对正确的患者管理至关重要，高级ONB与NEC的区别具有挑战性，需要额外的诊断标记。转录因子SATB2最近已被引入常规诊断中，作为远端肠分化的免疫组织化学标记。没有关于SN-NEN中SATB2和GATA3表达的具体数据。GATA3、SATB2和,为了比较，在一系列上皮和非上皮SN-NEN中研究CDX2表达。我们收集了在我院诊断和治疗的26例ONB和7例上皮SN-NEN。根据Hyams系统对ONBs进行分级，上皮NENs被重新分类为5个NEC，1MiNEN,和1个苯丙胺癌。使用标准自动化方案进行免疫组织化学。Hyams\'1-3级ONBs对SATB2进行了广泛而强烈的染色，而4级ONBs和NEC则整体阴性。MiNEN的非神经内分泌成分和两性癌呈强阳性。GATA3在Hyams1-3级ONB中具有异质性和不可预测的表达，而4级ONB和NEC完全阴性。CDX2在所有病例中均为阴性。我们的研究表明，第一次,SATB2和GATA3表达为Hyams\'1-3级ONB的特征，扩大了SATB2和GATA3阳性肿瘤的频谱，并表明Hyams\'4级ONBs不仅在临床上而且在生物学上与低分级ONBs不同。
