与Behçet病相关的肝细胞癌

S. Bouomrani, Mesfar Rim, Amri DhiaEddine, N. Belgacem, A. Hammami, W. Mahdhaoui, Ali Naffati
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摘要

肝细胞癌(HCC)在健康肝脏中的发生是罕见的,对临床医生来说是一个真正的诊断挑战。最近,几项研究报告,在贝氏病(BD)期间,癌症的风险特别增加。只有少数与这种血管炎相关的癌症零星病例被报道。我们报道了一名突尼斯患者在健康肝脏上发生非纤维板层HCC的原始观察结果,该患者随后接受了BD治疗。一名43岁的男子,自25岁起接受了BD随访,伴有孤立的皮肤和粘膜受累,并接受了秋水仙碱治疗,他因探索右疑病症的疼痛而入院,该疼痛自几个月以来一直在发展,以及夜热。临床检查发现肝脏肿大且疼痛。放射学检查(超声、计算机断层扫描(CT)和磁共振成像(MRI))显示一个巨大的肝脏肿瘤,动脉供血高度发达。超声引导下的肝细胞癌活检结束,无纤维板层型或肝硬化迹象。生物学测试没有异常,具体研究消除了潜在的慢性肝病(慢性乙型或丙型病毒性肝炎、血色素沉着症、Wilson病或自身免疫性肝炎)。患者因癌症晚期而接受了有症状的治疗。一个月后,由于多器官衰竭,他很快就去世了。健康肝脏的HCC是例外,BD被认为是一个可能的促成因素。因此,建议对任何BD患者,尤其是肝静脉血栓形成患者进行定期放射学监测。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Hepatocellular carcinoma associated to Behçet's disease
The occurrence of hepatocellular carcinoma (HCC) on a healthy liver is exceptional and represents a real diagnosis challenge for the clinician. Recently a particularly increased risk of cancer during Behc¸et’s disease (BD) was reported by several studies. Only a few sporadic cases of liver cancer associated with this vascultis have been reported. We report an original observation of non-fibrolamellar HCC occurring on healthy liver in a Tunisian patient followed for BD. A 43-year-old man, followed since the age of 25 for BD with isolated cutaneous and mucosal involvement, and treated by colchicine, was admitted for exploration of a pain of the right hypochondrium evolving since a few months associated with an important slimming, anorexia, and evening fever. The clinical examination noted a firm and painful hepatomegaly. Radiological exploration (ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI)) showed a bulky hepatic tumor with highly developed arterial blood supply. Ultrasound-guided biopsy concluded at HCC without signs of fibrolamellar type or cirrhosis. Biological tests were without abnormalities and specific investigations eliminated underlying chronic hepatopathy (chronic viral hepatitis B or C, hemochromatosis, Wilson’s disease or autoimmune hepatitis). The patient was treated symptomatically given the advanced stage of cancer. He quickly died after a month because of a multi-organ failure. HCC on healthy liver is exceptional and BD was suggested as a possible contributing factor. Thus, regular radiological monitoring seems to be recommended in any patient followed for BD, especially those with hepatic veins thrombosis.
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