Characteristics of hepatocellular carcinoma arising from Fontan-associated liver disease
Characteristics of hepatocellular carcinoma arising from Fontan-associated liver disease
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DOI：
10.1111/hepr.13500
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发表时间：
2020-04-30
影响因子：
4.2
通讯作者：
Tokushige, Katsutoshi
中科院分区：
文献类型：
--
作者：
Sagawa, Takaomi;Kogiso, Tomomi;Tokushige, Katsutoshi
目的肝细胞癌（HCC）可发生于Fontan相关性肝病（Fontan associated liver disease，FHD），称为FHD-HCC。HCC的临床特征尚不清楚。因此，我们研究了PD-HCC的发病率和临床特征。方法从1972年至2019年，122例患者在接受Fontan手术后发生肝脏疾病。12例（9.8%）肝硬化患者被诊断为肝细胞癌。结果Fontan手术后10年和20年的HCC发生率分别为0.8%和2.9%。患者诊断为HCC时的中位年龄为32.5岁（范围20.6-46.1岁），12例患者中有7例为男性。与非HCC患者相比，HCC患者肝硬化和多脾的发生率更高。8例患者的肝脏肿瘤被检测为单个结节，中位直径为47 mm（范围11-105 mm）。肝癌治疗手术切除2例，经导管动脉化疗栓塞或化疗3例，质子束治疗4例。3名患者因病情不佳无法治疗。4例患者死于肝/心力衰竭和HCC，3例患者的HCC得到控制。25年后生存率明显低于非肝癌患者（68.6% vs.97.9%，P < 0.01）。结论：122例原发性肝细胞癌患者中，12例在术后20年发展为肝癌。由于肝癌的并发症与预后不良有关，因此应在术后开始10年内对肝癌进行持续监测。
Aim Hepatocellular carcinoma (HCC) can arise from Fontan-associated liver disease (FALD); this is known as FALD-HCC. The clinical features of FALD-HCC are unclear. Thus, we examined the incidence and clinical characteristics of FALD-HCC.Methods From 1972 to 2019, 122 patients developed liver disease after undergoing Fontan procedures. HCC was diagnosed in 12 (9.8%) FALD patients. We compared FALD-HCC and non-HCC patients.Results The incidence of HCC was 0.8% and 2.9% in FALD 10 and 20 years after the Fontan procedure, respectively. The median age of patients at diagnosis of HCC was 32.5 years (range 20.6-46.1 years), and seven of the 12 patients were men. Patients with FALD-HCC had a higher incidence of liver cirrhosis and polysplenia than non-HCC patients. Liver tumors were detected as single nodules in eight patients, and the median diameter was 47 mm (range 11-105 mm). HCC was treated by surgical resection in two patients, transcatheter arterial chemoembolization or chemotherapy in three patients, and proton beam therapy in four patients. Three patients could not be treated because of their poor condition. Four patients died of liver/cardiac failure and HCC, and HCC was controlled in three patients. The survival rate after 25 years was significantly lower in patients with FALD-HCC than non-HCC patients (68.6% vs. 97.9%, respectively; P < 0.01).Conclusions: Of the 122 patients with FALD, 12 developed HCC 20 years after surgery. Because complications of HCC are associated with poor prognosis, constant surveillance for HCC should begin 10 years after surgery.