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病例报告:原发性肝副神经节瘤1例罕见病例:肝细胞癌的模仿者。

Case Report: A rare case of primary hepatic paraganglioma: a mimicker of hepatocellular carcinoma.

作者信息

Li Aitong, Ren Silu, Yang Xudan, Yang Chong, Lu Tao

机构信息

University of Electronic Science and Technology Hospital, Chengdu, Sichuan, China.

Sichuan Academy of Medical Sciences and Sichuan Provincial People's Hospital, Chengdu, China.

出版信息

Front Oncol. 2025 Sep 4;15:1570896. doi: 10.3389/fonc.2025.1570896. eCollection 2025.

DOI:10.3389/fonc.2025.1570896
PMID:40978045
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12444761/
Abstract

Paragangliomas (PGLs) are pheochromocytomas outside the adrenal glands, most commonly found in the retroperitoneal space, head and neck, bladder, and mediastinum. However, PGL occurring in the liver are extremely rare. We present a case of a 70-year-old woman who presented to our hospital with right upper abdominal pain, persisting for 2 years. Abdominal contrast-enhanced computed tomography (CT) revealed a 3.8 x 2.7 cm hypervascular nodule in the caudate lobe of the liver, demonstrating arterial phase hyperenhancement and portal/delayed phase washout. Magnetic resonance imaging (MRI) further demonstrated diffusion restriction and low signal intensity in the hepatobiliary phase (HBP) of the nodule. Based on these imaging features, hepatocellular carcinoma (HCC) was initially diagnosed radiologically. Surgical resection was performed, and immunohistochemical staining revealed positivity for chromogranin A (CgA), synaptophysin (Syn), and S - 100 protein, confirming the diagnosis of primary hepatic PGL (HPGL). This case highlights that hypervascular lesions with washout and HBP hypointensity may mimic HCC. Pathological verification is crucial, especially in patients without typical HCC risk factors. Although exceedingly rare, HPGL should be considered in the differential diagnosis of hypervascular hepatic nodules demonstrating typical arterial phase hyperenhancement and portal/delayed washout on CT/MRI, particularly in female patients presenting with nonspecific symptoms and lacking typical HCC risk factors such as hepatitis, alcohol abuse, or elevated tumor markers.

摘要

副神经节瘤(PGLs)是肾上腺外的嗜铬细胞瘤,最常见于腹膜后间隙、头颈部、膀胱和纵隔。然而,发生在肝脏的PGL极为罕见。我们报告一例70岁女性,因右上腹疼痛2年就诊于我院。腹部增强计算机断层扫描(CT)显示肝脏尾状叶有一个3.8×2.7 cm的高血管结节,动脉期强化明显,门静脉/延迟期廓清。磁共振成像(MRI)进一步显示该结节在肝胆期(HBP)有扩散受限和低信号强度。基于这些影像学特征,最初通过放射学诊断为肝细胞癌(HCC)。进行了手术切除,免疫组织化学染色显示嗜铬粒蛋白A(CgA)、突触素(Syn)和S - 100蛋白呈阳性,确诊为原发性肝副神经节瘤(HPGL)。该病例强调,有廓清和HBP低信号的高血管病变可能酷似HCC。病理证实至关重要,尤其是对于没有典型HCC危险因素的患者。尽管极为罕见,但在CT/MRI上表现为典型动脉期强化和门静脉/延迟期廓清的高血管性肝结节的鉴别诊断中,应考虑HPGL,特别是对于出现非特异性症状且缺乏肝炎、酗酒或肿瘤标志物升高等典型HCC危险因素的女性患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5e9abbd2f1b3/fonc-15-1570896-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/caca807234e5/fonc-15-1570896-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5352b7ebf548/fonc-15-1570896-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5654f5e1801f/fonc-15-1570896-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5e9abbd2f1b3/fonc-15-1570896-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/caca807234e5/fonc-15-1570896-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5352b7ebf548/fonc-15-1570896-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5654f5e1801f/fonc-15-1570896-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ba37/12444761/5e9abbd2f1b3/fonc-15-1570896-g004.jpg

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本文引用的文献

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Pheochromocytoma: a changing perspective and current concepts.嗜铬细胞瘤:不断变化的观点与当前概念
Ther Adv Endocrinol Metab. 2023 Oct 29;14:20420188231207544. doi: 10.1177/20420188231207544. eCollection 2023.
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An Insight Into the Role of Alpha-Fetoprotein (AFP) in the Development and Progression of Hepatocellular Carcinoma.浅析甲胎蛋白(AFP)在肝细胞癌发生发展中的作用。
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