Epidemiological study of histologically proven advanced hepatocholangiocarcinoma: An AGEO multicenter retrospective study

Abstract

Background: Hepatocholangiocarcinoma (cHCC-ICC) is a rare primary hepatic tumor combining the features of cholangiocarcinoma and hepatocellular carcinoma. Few data about the epidemiology of cHCC-ICC have been reported, mainly from surgical series in Asian and American populations. The aim of this study was to evaluate epidemiological features and overall survival (OS) of histologically proven advanced cHCC-ICC patients. Methods: Data from patients treated for histologically proven cHCC-ICC in six French university hospitals between 2008 and February 2017, were retrospectively collected. The main clinical, biological, treatment and follow up data were reported. Statistical analysis was performed using Graph Pad Prism 6. Results: Thirty patients were included (76.6% of men, median age 64 years [extreme 37-88]). Cirrhosis was associated in 33.3% of cases (Child-Pugh score A: 70%). Positive serology for hepatitis B virus and C was found in respectively, 5 (16.6%) and 2 (6.6%) patients; with 1 co-infection. Chronic alcoholism was noted in 33.3%, diabetes and obesity were both present in 26.6%of cases. Alpha-fetoprotein, carbohydrate antigen 19-9 and carcinoembryonnic antigen serum levels were above normal in respectively 39% (median = 5.3 μg/L [2 - 21 479]), 50%(median = 21.8 IU/ mL [4.5-20 000]) and 14%(median = 2.4 μg/L [2-88]) of cases. Six patients (20%) were initially treated by surgical resection. At the diagnosis of advanced disease, 66.6% of patients hadmultifocal hepatic lesions, 50% distant metastases (bone (23.3%), lung (20%), peritoneal metastases (13.3%)). Twenty-seven patients (90%) received first line of systemic treatment. Twenty-four patients were treated by chemotherapy: Gemcitabine (Gem) alone (n=1), Gem+oxaliplatin (Gemox) (n=12), Gemox + bevacizumab (n =9), Gem+cisplatin (n=2). Two patients received chemoembolization, 1 patient received sorafenib. Twenty-one (70%) and 4 (13.3%) patients had a second and third line of treatment, respectively.Median OS was 14.5months. Conclusions: Advanced cHCC-ICC appear to be aggressive tumors with a poor prognosis. Cirrhosis was associated in one third of cases. Systemic treatments are not standardized and must be evaluated in a dedicated study.