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原发性肝神经内分泌肿瘤酷似破裂的肝细胞癌伴甲胎蛋白升高:病例报告及文献复习

Primary Hepatic Neuroendocrine Tumor Mimicking Ruptured Hepatocellular Carcinoma with AFP Elevation: A Case Report and Literature Review.

作者信息

Huang Hai-Feng, Jin Piao-Piao, Yang Han-Jin, Zhang Chun-Jun, Zhang Xin, Wang Jun-Sen, Yu Jia-Jie, Zhang Bo, Zhang Yun, Hu Qi-da

机构信息

Department of Surgery, Shengzhou People's Hospital, Shengzhou 312400, People's Republic of China.

Health Management Center, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, People's Republic of China.

出版信息

Onco Targets Ther. 2020 Feb 3;13:975-979. doi: 10.2147/OTT.S236728. eCollection 2020.

Abstract

Liver cancer is a common malignant disease in China, while the primary hepatic neuroendocrine tumor (PHNET) is extremely rare presented with various manifestations. We herein describe an interesting PHNET case, which was clinically diagnosed as hepatocellular carcinoma (HCC) based on strong clinical evidence and the national guideline, but confirmed to be PHNET by pathology. A42-year-old Chinese male was admitted for persistent upper abdominal pain, and CT scan revealed a huge liver tumor in the left lobe. The tumor presented attributes of tumor rupture, portal vein tumor thrombus, elevated serum AFP level, background hepatitis B virus infection history, and radiological features mimicking typical HCC. After left semi-hepatectomy was performed for curative treatment of the primary "HCC", the pathology demonstrated the correct diagnosis be poorly differentiated neuroendocrine carcinoma (NEC). The immunohistochemistry assays showed positive neuroendocrine markers of CgA and Syn and negative HCC markers of Hep Par 1 and GPC3, ruling out concurrent HCC. This case and literature review suggest that in spite of rare incidence, PHNET should be considered as a possible diagnosis when lacking a confirmative pathology result, even when sufficient evidence of typical presentation exist to establish the clinical diagnosis of HCC.

摘要

肝癌是中国常见的恶性疾病,而原发性肝脏神经内分泌肿瘤(PHNET)极为罕见,临床表现多样。我们在此描述一例有趣的PHNET病例,该病例基于有力的临床证据和国家指南在临床上被诊断为肝细胞癌(HCC),但病理检查确诊为PHNET。一名42岁的中国男性因持续性上腹部疼痛入院,CT扫描显示左叶有一个巨大的肝脏肿瘤。该肿瘤具有肿瘤破裂、门静脉瘤栓、血清甲胎蛋白水平升高、乙肝病毒感染史背景以及类似典型HCC的影像学特征。在对原发性“HCC”进行左半肝切除以进行根治性治疗后,病理结果显示正确诊断为低分化神经内分泌癌(NEC)。免疫组化检测显示嗜铬粒蛋白A(CgA)和突触素(Syn)神经内分泌标记物呈阳性,而肝细胞癌标记物Hep Par 1和磷脂酰肌醇蛋白聚糖3(GPC3)呈阴性,排除了合并HCC。该病例及文献回顾表明,尽管PHNET发病率很低,但在缺乏确诊病理结果时,即使存在足以确立HCC临床诊断的典型表现证据,也应考虑将PHNET作为一种可能的诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3d12/7007302/6fbe532953f0/OTT-13-975-g0001.jpg

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