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肝脏中发生的具有神经内分泌分化的肿瘤的临床病理特征。

Clinicopathological features of neoplasms with neuroendocrine differentiation occurring in the liver.

作者信息

Nomura Yoriko, Nakashima Osamu, Akiba Jun, Ogasawara Sachiko, Fukutomi Shogo, Yamaguchi Rin, Kusano Hironori, Kage Masayoshi, Okuda Koji, Yano Hirohisa

机构信息

Department of Diagnostic Pathology, Kurume University Hospital, Kurume, Fukuoka, Japan.

Department of Surgery, Kurume University School of Medicine, Kurume, Fukuoka, Japan.

出版信息

J Clin Pathol. 2017 Jul;70(7):563-570. doi: 10.1136/jclinpath-2016-203941. Epub 2016 Nov 23.

Abstract

BACKGROUND/AIMS: We investigated the clinicopathological features of hepatic neuroendocrine tumours (NET) and neuroendocrine carcinoma (NEC), which remain largely unknown.

MATERIAL AND METHODS

We examined 1235 tumours from 1048 patients who had undergone curative hepatectomy for liver neoplasms at Kurume University Hospital. Pathological diagnoses were based on the 2010 WHO Classification of Tumours of the Digestive System. We performed immunostaining for hepatocyte markers (eg, hepatocyte paraffin (HepPar)-1), neuroendocrine markers (eg, chromogranin A (CGA)) and the proliferation marker (Ki-67).

RESULTS

There were four cases of NET G2 (0.38%) and five of hepatic malignant tumours with an NEC component (HNEC) (0.48%). HNEC cases were classified into three types, that is, transitional, intermediate and separate types, according to their histological and immunohistochemical features. In the former two types, the NEC component intermingled with the moderately to poorly differentiated hepatocellular carcinoma (HCC) component or intermediate component consisting of tumour cells showing the colocalisation of CGA and HepPar-1. In the separate type, the NEC and poorly differentiated HCC components were present separately, whereas the sarcomatous HCC component was detected in the vicinity of the NEC component. Ki-67 labelling indices of the NET G2, HCC and NEC components of HNEC were 6.8%, 14.9% and 58.9%, respectively.

CONCLUSIONS

Primary hepatic NET and NEC are very rare tumours. The NEC component in HNEC showed high proliferative activity and influenced patient prognoses.

摘要

背景/目的:我们研究了肝神经内分泌肿瘤(NET)和神经内分泌癌(NEC)的临床病理特征,目前这些特征在很大程度上仍不清楚。

材料与方法

我们检查了来自久留米大学医院因肝脏肿瘤接受根治性肝切除术的1048例患者的1235个肿瘤。病理诊断依据2010年世界卫生组织消化系统肿瘤分类。我们对肝细胞标志物(如肝细胞石蜡(HepPar)-1)、神经内分泌标志物(如嗜铬粒蛋白A(CGA))和增殖标志物(Ki-67)进行了免疫染色。

结果

有4例G2级NET(0.38%)和5例具有NEC成分的肝恶性肿瘤(HNEC)(0.48%)。根据组织学和免疫组化特征,HNEC病例分为三种类型,即过渡型、中间型和分离型。在前两种类型中,NEC成分与中分化至低分化肝细胞癌(HCC)成分或由显示CGA和HepPar-1共定位的肿瘤细胞组成的中间成分混合。在分离型中,NEC和低分化HCC成分分别存在,而在NEC成分附近检测到肉瘤样HCC成分。NET G2、HCC和HNEC的NEC成分的Ki-67标记指数分别为6.8%、14.9%和58.9%。

结论

原发性肝NET和NEC是非常罕见的肿瘤。HNEC中的NEC成分显示出高增殖活性并影响患者预后。

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