El-Serag Hashem B, Davila Jessica A
Section of Health Services Research, The Houston Veterans Affairs Medical Center and Baylor College of Medicine, 2002 Holcombe Boulevard (152), Houston, TX 77030, USA.
Hepatology. 2004 Mar;39(3):798-803. doi: 10.1002/hep.20096.
There have been no population-based studies of the epidemiology and prognosis of patients with fibrolamellar carcinoma (FLC). We conducted a retrospective cohort study using information collected by population-based registries of the Surveillance, Epidemiology, and End Results (SEER) program. The demographic features, stage at diagnosis, and type of therapy, as well as age-adjusted incidence rates and observed and relative survival rates were compared between persons with FLC and those with hepatocellular carcinoma (HCC) diagnosed between 1986 and 1999. A multivariate Cox proportional hazards model was constructed to examine the effect of histology (FLC vs. HCC) on the risk of mortality. There were 68 microscopically confirmed cases of FLC and 7,896 cases of HCC. FLC constituted 0.85% of all cases of primary liver cancer and 13.4% of all cases below the age of 40. Compared to HCC, patients with FLC were more likely to be younger (mean age 39 vs. 65), female (51.5% vs. 26.3%), and white (85.3% vs. 56.9%). A greater proportion of case with FLC had localized disease (41.2% vs. 30.9%), or received potentially curative therapy (resection, transplantation), compared to cases with HCC. The age-adjusted incidence rate for FLC was 0.02 per 100,000; No significant differences in age-adjusted incidence rates were observed by gender or race. The 1- and 5-year observed and relative survival rates were significantly longer in patients with FLC than HCC. The 5-year relative survival rate was 31.8% (95% CI, 20.5%-43.1%) for FLC, compared with 6.8% (95% CI, 6.3 %-7.4 %) for HCC. Adjusting for differences in age, gender, race, stage of disease, receipt of resection or transplantation, and time of diagnosis, FLC was independently associated with a 46% reduction in risk of mortality within 5 years compared with HCC. In conclusion, in a population-based study, we observed remarkable differences in the epidemiology and prognosis of FLC compared to HCC.
目前尚无基于人群的纤维板层癌（FLC）患者流行病学及预后研究。我们利用监测、流行病学及最终结果（SEER）计划基于人群的登记处收集的信息进行了一项回顾性队列研究。比较了1986年至1999年间诊断为FLC的患者与肝细胞癌（HCC）患者的人口统计学特征、诊断时的分期、治疗类型，以及年龄调整发病率、观察到的生存率和相对生存率。构建了多变量Cox比例风险模型，以检验组织学类型（FLC与HCC）对死亡风险的影响。共有68例经显微镜确诊的FLC病例和7896例HCC病例。FLC占原发性肝癌所有病例的0.85%，占40岁以下所有病例的13.4%。与HCC相比，FLC患者更可能较年轻（平均年龄39岁对65岁）、女性（51.5%对26.3%）、白人（85.3%对56.9%）。与HCC病例相比，FLC病例中更大比例为局限性疾病（41.2%对30.9%），或接受了可能治愈性的治疗（切除、移植）。FLC的年龄调整发病率为每10万人0.02；未观察到按性别或种族划分的年龄调整发病率有显著差异。FLC患者的1年和5年观察生存率及相对生存率显著长于HCC患者。FLC的5年相对生存率为31.8%（95%CI，20.5%-43.1%），而HCC为6.8%（95%CI，6.3%-7.4%）。在调整年龄、性别、种族、疾病分期、是否接受切除或移植以及诊断时间的差异后，与HCC相比，FLC与5年内死亡风险独立降低46%相关。总之，在一项基于人群的研究中，我们观察到FLC与HCC在流行病学和预后方面存在显著差异。
