Department of Surgery, Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD 21287, USA.
J Am Coll Surg. 2012 Dec;215(6):820-30. doi: 10.1016/j.jamcollsurg.2012.08.001. Epub 2012 Sep 13.
Fibrolamellar hepatocellular carcinoma (FLC) is a rare primary liver tumor presenting earlier in life than nonfibrolamellar hepatocellular carcinoma (NFL-HCC), with distinct epidemiologic and clinical characteristics. Although FLC is believed to have a better prognosis than NFL-HCC, data on treatment and prognosis are scarce. We performed a systematic review to investigate treatment options and clinical outcomes of patients with FLC.
The study is a systematic review of the literature and pooled analysis of individual patient data.
A total of 35 series were analyzed, reporting on 575 patients (52% female, elevated alpha-fetoprotein in 10%, cirrhosis in 3%, hepatitis B in 2%), most of whom were treated with partial hepatectomy (55%) or orthotopic liver transplantation (23%). Nineteen studies provided data on 206 individual patients with a median age of 21 years and tumor size of 12 cm. Median overall survival (OS) was 39 months; 1-year, 3-year, and 5-year OS rates were 85%, 53%, and 44%, respectively. For patients treated with liver resection, median OS was 18.5 years and 1-year, 3-year, and 5-year OS were 93%, 80%, and 70%, respectively. Based on data from 15 studies, FLC appeared to follow a relatively indolent course compared with NFL-HCC.
Patients with FLC treated with partial hepatectomy have excellent long-term survival, with 5-year overall survival reaching 70%. Patients fared worse with the use of other therapeutic options including chemotherapy, intra-arterial therapy, and transplantation, although data directly comparing resection vs transplantation were limited.
纤维板层肝细胞癌（FLC）是一种罕见的原发性肝癌，比非纤维板层肝细胞癌（NFL-HCC）更早发生，具有独特的流行病学和临床特征。尽管人们认为 FLC 的预后优于 NFL-HCC，但关于治疗和预后的数据仍然有限。我们进行了一项系统综述，以调查 FLC 患者的治疗选择和临床结局。
本研究是对文献的系统综述和个体患者数据的汇总分析。
共分析了 35 个系列，报告了 575 例患者（52%为女性，10%甲胎蛋白升高，3%肝硬化，2%乙型肝炎），其中大多数患者接受了部分肝切除术（55%）或原位肝移植（23%）。19 项研究提供了 206 例患者的个体数据，中位年龄为 21 岁，肿瘤大小为 12cm。中位总生存期（OS）为 39 个月；1 年、3 年和 5 年 OS 率分别为 85%、53%和 44%。接受肝切除术治疗的患者中位 OS 为 18.5 年，1 年、3 年和 5 年 OS 率分别为 93%、80%和 70%。基于 15 项研究的数据，FLC 似乎比 NFL-HCC 表现出相对惰性的病程。
接受部分肝切除术治疗的 FLC 患者具有出色的长期生存，5 年总生存率达到 70%。其他治疗选择，包括化疗、肝动脉治疗和移植，患者预后较差，尽管直接比较切除术与移植术的数据有限。
