来自：双语学影像；本期病例选自AuntMinnie.com
Our appreciation is extended to Dr. James Chen, University of Pennsylvania Department of Radiology, for contributing this case.
History and Radiographs
A 22-year-old woman presents with several months of abdominal pain. She is found to have hepatomegaly on physical examination.
22岁女性，腹痛数月，体检发现肝脏肿大。
1.The lesion demonstrates a T1 and T2 hypointense central scar.
True
False
2.The liver contour is nodular, consistent with changes of cirrhosis.
True
False
3.The lesion demonstrates peripheral nodular enhancement on early arterial phase imaging.
True
False
4.The lesion demonstrates areas of necrosis.
True
False
5.Which of the following is the most likely associated with the etiology of the above lesion?
History of alcohol abuse
Chronic hepatitis C
Oral contraceptive use
Hepatic steatosis
None of the above
6.Which of the following is the most likely diagnosis for this mass?
Focal nodular hyperplasia
Cavernous hemangioma
Peripheral cholangiocarcinoma
Hepatic adenoma
Fibrolamellar hepatocellular carcinoma (FLC)
Additional Questions
7.The central scar in FLC can be differentiated from that of focal nodular hyperplasia (FNH) based on which of the following?
FNH scar is T1 hyperintense
FNH scar is T2 hyperintense
FLC scar is T1 hyperintense
FLC scar is T2 hyperintense
8.In patients with the above mass, which of the following lab markers is elevated in the majority of cases?
AFP
CA 19-9
CEA
Chromogranin
None of the above
9.The preferred treatment for this lesion is which of the following?
Surgical resection
Percutaneous thermal ablation
Chemotherapy
Stereotactic radiosurgery
Immunotherapy
True
False
False
True
None of the above
Fibrolamellar hepatocellular carcinoma (FLC)
FNH scar is T2 hyperintense
None of the above
Surgical resection
There is a large heterogeneous mass centered in the posterior right hepatic lobe, which demonstrates T2 hyperintensity, mild T1 hypointensity, and a T1 and T2 hypointense central scar. The background liverparenchyma does not demonstrate findings of cirrhosis.
肝脏右后叶巨大不均质肿块，T2高信号，T1稍低信号，肿块可见中央瘢痕，T1WI、T2WI均呈低信号。肝实质未见明显肝硬化征象。
On postgadolinium images, the mass demonstrates brisk heterogeneous enhancement in the early arterial phase with more homogeneous enhancement by the delayed phase. The central scar enhances by delayed-phase imaging, but does not significantly enhance in early arterialphase. There are nonenhancing, T1 hypointense regions throughout the mass corresponding to areas of necrosis.
增强扫描，肿块动脉早期明显强化，强化信号不均匀。延迟期强化信号趋向均匀。病灶中央瘢痕早期无明显强化，延迟强化。肿块内可见T1WI低信号区、增强扫描无强化，对应肿块内坏死区。
Diffusion-weighted imaging demonstrates area of restricted diffusion within the right hepatic lobe lesion and a separate smaller lesion in the left hepatic lobe.
DWI示肝右叶肿块弥散受限。肝左叶可见一孤立性小病灶。
Diagnosis
Fibrolamellar hepatocellular carcinoma
纤维板层样肝癌
Key Points
No specific risk factors: Usually not associated with underlying cirrhosisor liver disease, but cirrhosis may be present in small minority of cases (<>
无明显特殊致病因素：一般与肝硬化无直接关系，但少数患者可伴有肝硬化病史。
Slow-growing tumor:However, can frequently present with metastatic disease.
肿瘤生长缓慢，但常出现转移灶。
Peak age: 20s to 30s (mean: 23 years old). Consist of 35% of all HCC in patients < 50="" years="">
好发年龄：20-30岁（平均年龄23岁）。而35%的HCC患者年龄在50岁以下。
No gender predilection.
无明显性别差异
Caucasian predilection.
白种人较多见。
Symptoms include pain, hepatomegaly, palpable mass, cachexia, fever, and gynecomastia.
常见症状包括：腹痛、肝脏肿大、可触及包块、恶病质、发热、男性乳房发育。
Imaging Features
Satellite nodules often present.
常可见卫星灶。
Central scar: 20% to 71% cases, stellate or amorphous conformation.
约20%-71%的患者可见中央瘢痕，一般为星芒状或不定形状。
Locoregional lymphadenopathy in 50% cases.
约50%的患者可见局部淋巴结肿大
T1-weighted images:
Mass: Heterogeneous, mild hypointensity
肿块表现为不均匀略低信号
Central scar and septa: Hypointense
中央瘢痕为低信号
T2-weighted images:
Mass: Heterogeneous, hyperintense
肿块表现为不均匀高信号
Central scar and septa: Hypointense (important distinguishing factor from FNH, which has a T2 hyperintense central scar)
中央瘢痕及分隔为低信号（FNH中央瘢痕T2WI高信号，这是两者重要的鉴别点）
Postgadolinium images in arterial and portal phases:
Mass:
Intense heterogeneous enhancement during arterial and portal phases
Homogeneous enhancement by delayed phase
肿块于动脉期、门静脉期明显不均匀强化，延迟期均匀强化。
Scar and septa: Delayed enhancement
瘢痕及分隔呈延迟强化。
Additional features that can be variably present 其他征象
Calcification 钙化
Necrosis 坏死
Metastatic lymphadenopathy 转移性淋巴结肿大
Satellite nodules 卫星结节
Important to distinguish from the following 主要鉴别诊断
FNH: Can mimic FLC due to central scar.
局灶性结节增生：由于中央瘢痕而易与本病混淆。
Conventional hepatocellular carcinoma (HCC): Can be difficult to differentiate on imaging alone; tissue diagnosis often required.
肝细胞肝癌：单纯依靠影像学有时难以鉴别，往往需要组织学诊断。
Treatment
Surgical resection of hepatic lesion and regional nodes -- resectable in 48% of cases
约48%的患者可采用手术方法治疗，切除肝脏肿块及局部淋巴结。
Chemotherapy and/or radiation for inoperable disease
对于无法手术的患者，可采用放化疗治疗。
Prognosis
Traditionally thought to be superior to conventional HCC, but recent studies have shown contradictory outcome data when stratifying underlying cirrhosis.
传统上认为纤维板层样肝癌预后好于传统HCC，但最近的研究表明，如果合并有肝硬化基础时，结果恰恰相反。
Five-year survival is 50% to 75% for resectable disease, but much lower for unresectable disease.
对于可手术的患者，5年生存率约50%-75%；无法手术的患者，5年生存率明显较低。
联系客服
