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肝脏原发性神经内分泌癌与肝细胞癌合并存在

Combined primary neuroendocrine carcinoma and hepatocellular carcinoma of the liver.

作者信息

Yang Chii-Shuenn, Wen Mei-Chin, Jan Yee-Jee, Wang John, Wu Cheng-Chung

机构信息

Department of Pathology, Taichung Veterans General Hospital, Taichung, Taiwan, R.O.C.

出版信息

J Chin Med Assoc. 2009 Aug;72(8):430-3. doi: 10.1016/S1726-4901(09)70400-9.

DOI:10.1016/S1726-4901(09)70400-9
PMID:19686999
Abstract

We report a unique case of combined primary neuroendocrine carcinoma (NEC) and hepatocellular carcinoma (HCC) of the liver in a 65-year-old male patient. The patient underwent segmental resection of the liver and regional lymph node dissection for a tumor mass that measured 7.5 cm in diameter in the right lobe, with regional lymphadenopathy. Histologically, the hepatic tumor was composed of predominantly small-cell NEC, but admixed with a small island of moderately differentiated HCC. We speculate that the NEC originated from a poorly differentiated tumor clone of an HCC that underwent neuroendocrine differentiation, and that this tumor was now at the end stage of the transitional period from HCC to NEC, based on the small amount of disappearing HCC. Ki-67 and p53 expression were higher in the NEC than in the HCC, and the lymph nodes showed only metastatic NEC. Therefore, this kind of tumor had a more aggressive clinical course in accordance with being an NEC rather than a conventional HCC. Three months after operation, the patient had multiple recurrent tumor nodules within the liver, spreading the metastasis to the adrenal glands and para-aortic lymph nodes. The patient died 1 year after operation.

摘要

我们报告了一例独特病例,一名65岁男性患者患有肝脏原发性神经内分泌癌(NEC)和肝细胞癌(HCC)合并症。患者因右叶直径7.5 cm的肿瘤肿块伴区域淋巴结肿大,接受了肝脏段切除术和区域淋巴结清扫术。组织学检查显示,肝脏肿瘤主要由小细胞NEC组成,但混有一小片中度分化的HCC。我们推测,NEC起源于发生神经内分泌分化的HCC低分化肿瘤克隆,基于少量逐渐消失的HCC,该肿瘤目前正处于从HCC向NEC过渡的末期。NEC中Ki-67和p53表达高于HCC,且淋巴结仅显示转移性NEC。因此,这种肿瘤作为NEC,其临床病程比传统HCC更具侵袭性。术后三个月,患者肝脏内出现多个复发性肿瘤结节,并转移至肾上腺和主动脉旁淋巴结。患者术后1年死亡。

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