摘要目的 探讨肝脏原发性神经内分泌肿瘤(PHNEN)的临床病理学特征,分级标准及预后相关指标.方法 收集复旦大学附属中山医院11年间确诊的35例PHNEN标本(其中32例病理资料完整,可进行组织病理分析),按消化系统肿瘤WHO分类(2010)神经内分泌肿瘤分级标准将PHNEN分为神经内分泌瘤(NET) G1、NET G2、神经内分泌癌(NEC)；并将NET( G1,G2)与NEC作为2个组,评估肿瘤大小、肿瘤组织分化程度、生长方式、坏死、瘤栓、核分级、核分裂象以及Ki-67阳性指数8项指标在两组间分布情况,同时探讨8项指标与PHNEN预后的关系.结果 32例PHNEN分为NET G1(1例),NET G2(14例),NEC(17例)；2组在肿瘤组织分化程度、坏死、核分级、核分裂象以及Ki-67阳性指数5个指标的比较上,分布存在差异(P＜0.05)；而肿瘤大小、生长方式、有无瘤栓的差异不明确(P＞0.05).肿瘤组织分化程度、生长方式、坏死、核分级、核分裂象以及Ki-67阳性指数均可影响生存率(P＜0.05),而肿瘤大小和有无瘤栓对生存率影响价值有限(P＞0.05).结论 消化系统神经内分泌肿瘤WHO分类(2010)可以协助预测PHNEN的恶性程度及判断预后,具有可行性；肿瘤组织分化差、浸润性生长、有坏死、核分裂象多、核分级高、Ki-67高表达均是PHNEN预后不良的指标.

abstractsObjective To study the clinicopathologic features,criteria for grading and prognostic factors of primary hepatic neuroendocrine neoplasms. Methods Thirty-five cases of primary hepatic neuroendocrine neoplasm were retrieved from the archival files over a period of 11 years (with 32 cases having integrated data).According to the 2010 WHO classification of tumors of the digestive system,the cases were categorized into three groups:neuroendocrine tumor grade 1 ( NET G1 ),neuroendocrine tumor grade 2 (NET G2) and neuroendocrine carcinoma (NEC).Statistical correlation between various histologic parameters and survival data was analyzed.Results Statistical analysis showed significant difference between NET [G1 ( 1 case)/G2 ( 14 cases)] and NEC ( 17 cases) groups in terms of tumor differentiation,necrosis,nuclear atypia,mitotic count and Ki-67 proliferative index (P ＜ 0.05).There was no statistically significant difference in tumor size,growth pattern and presence of vascular tumor emboli (P ＞ 0.05).The survival rate of patients correlated with tumor differentiation,growth pattern,necrosis,nuclear atypia,mitotic count and proliferative index (P ＜ 0.05 ).There was no statistically significant difference between patient survival and tumor size or presence of vascular tumor emboli ( P ＞ 0.05 ). Conclusions The subdivision of primary hepatic neuroendocrine neoplasm according to the 2010 WHO classification of tumors of the digestive system helps to evaluate the malignant potential and prognosis of the tumors.Prognostically useful histologic parameters include tumor differentiation,growth pattern,necrosis,nuclear atypia,mitotic count and proliferative index.