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Abstract：
BACKGROUND: Hepatocellular carcinoma (HCC) is a primary tumor of the liver. The majority of HCCs are associated most frequently with chronic B or C viral hepatitis, alcohol intake or aflatoxin exposure. Cirrhosis is a strong risk factor associated with HCC. The causes of liver cirrhosis are chronic viral hepatitis, alcohol intake, metabolic diseases (NAFLD), hemocromathosis, alfa 1 antitrypsisn deficiency. All aetiologic forms of cirrhosis are at risk to be complicated by HCC development, but the risk is higher for patients diagnosed with chronic viral hepatitis. Comparing to the above-mentioned causes, PBC and AIH are less associated with the risk of HCC development. A 71-year old Caucasian female previously diagnosed with overlap syndrome (AIH type 1 and PBC-ANA, SMA and AMA antibodies positive), liver cirrhosis, a nodule in the VI/VIIth hepatic segment, systemic sclerosis sine scleroderma, Hashimoto\'s thyroiditis, antiphospholipid syndrome, gastric antral vascular ectasia (GAVE) (with 2 previous sessions of argon plasma coagulation), cholecystectomy, arterial hypertension and nephro-angiosclerosis presented to the 2nd Department of Internal Medicine in Cluj-Napoca for a follow-up. The patient was following treatment with UDCA (Ursodeoxycholic acid), azathioprine, Plaquenil, calcium channel blockers, angiotensin-converting-enzyme inhibitor, calcium and vitamin D supplementation. The abdominal ultrasound showed a subcapsular hypoechoic nodule with a diameter of 29 mm (at the moment of the diagnosis the diameter was 9/10 mm) in the VI/VIIth hepatic segment. The contrast-enhanced ultrasound (CEUS) characterised the nodule as specific for hepatocellular carcinoma (LI-RADS 5). On MRI with gadoxetate disodium the nodule was hypovascular, non-specific, being classified as LI-RADS 3. An atypical resection of the VIIth hepatic segment was performed and the histohistological examination and imunohistochemistry (Hep Par-a positive, Glypican3 positive, CD34 positive) revealed a moderately differentiated hepatocellular carcinoma (G2), pT2 N0 M0 L0 V1 R0.
CONCLUSIONS: Autoimmune hepatitis, PBC and the overlap syndrome are less associated with the development of liver cirrhosis and HCC than other chronic liver diseases, especially if other risk factors are not associated. This case highlights the importance of a proper surveillance of cirrhotic patients every 6 months including abdominal ultrasound and AFP levels is crucial for an early diagnosis of a HCC.
CONCLUSIONS: Autoimmune hepatitis, PBC and the overlap syndrome are less associated with the development of liver cirrhosis and HCC than other chronic liver diseases, especially if other risk factors are not associated. This case highlights the importance of a proper surveillance of cirrhotic patients every 6 months including abdominal ultrasound and AFP levels is crucial for an early diagnosis of a HCC.
摘要：
背景：肝细胞癌（HCC）是肝脏的原发性肿瘤。大多数的肝癌是最常见的与慢性乙型或丙型病毒性肝炎，酒精摄入或黄曲霉毒素暴露。肝硬化是与HCC相关的强危险因素。肝硬化的病因是慢性病毒性肝炎,酒精摄入量,代谢性疾病（NAFLD），血石病,α1抗胰蛋白酶缺乏症。所有病因形式的肝硬化都有可能因HCC发展而复杂化，但是对于被诊断为慢性病毒性肝炎的患者，风险更高。与上述原因相比，PBC和AIH与HCC发展的风险较小。一名71岁的白人女性，先前被诊断患有重叠综合征（AIH1型和PBC-ANA，SMA和AMA抗体阳性)，肝硬化,VI/VII肝段的结节，系统性硬化症，硬皮病，桥本甲状腺炎,抗磷脂综合征,胃窦血管扩张（GAVE）（前2次氩离子凝固术），胆囊切除术,向Cluj-Napoca的第二内科就诊的动脉高血压和肾血管硬化进行随访。患者接受UDCA（熊去氧胆酸）治疗，硫唑嘌呤，Plaquenil,钙通道阻滞剂，血管紧张素转换酶抑制剂，补充钙和维生素D。腹部超声显示VI/VIII肝段有直径29mm（诊断时直径为9/10mm）的包膜下低回声结节。对比增强超声（CEUS）将结节表征为肝细胞癌（LI-RADS5）。在使用gadoxetate二钠的MRI上，结节为血管不足，非特异性,被分类为LI-RADS3。对第VII个肝段进行了非典型切除，并进行了组织组织学检查和免疫组织化学（HepPar-a阳性，Glypican3阳性,CD34阳性）显示为中分化肝细胞癌（G2），pT2N0M0L0V1R0。
结论：自身免疫性肝炎，与其他慢性肝病相比，PBC和重叠综合征与肝硬化和HCC的发展较少相关。特别是如果其他风险因素没有关联。该病例强调了每6个月对肝硬化患者进行适当监测的重要性，包括腹部超声和AFP水平对于HCC的早期诊断至关重要。
结论：自身免疫性肝炎，与其他慢性肝病相比，PBC和重叠综合征与肝硬化和HCC的发展较少相关。特别是如果其他风险因素没有关联。该病例强调了每6个月对肝硬化患者进行适当监测的重要性，包括腹部超声和AFP水平对于HCC的早期诊断至关重要。
