gms | German Medical Science

Background: Intrahepatic cholangiocarcinoma (ICC) is a rare entity. In the western world the incidence is below one in 100.000. Yet, there has been a sharp increase over the past 30 years, more than doubling the incidence. Diagnosis is often delayed due to oligosymptomatic growth. Survival is poor and non surgical treatment options are limited. Surgical resection is the only chance for cure. In order to select good surgical candidates, robust prognostic factors are needed.

Materials and methods: From a prospective database of all liver resections at a university liver center 79 patients were identified from 2001-2015, excluding all gallbladder carcinomas and extrahepatic tumors of the biliary tract such as “Klatskin” tumors or distal bile duct carcinomas. Irresectable tumors, which were only biopsied, were also excluded. Actuarial survival was estimated by the Kaplan-Meier method. Multivariate survival analysis was performed using the Cox proportional hazard model.

Results: 54% of patients were male. Median age was 65 years (33-83). 81% had major hepatectomies (hemihepatectomy or >4 segments). 29 % had multicentric tumors. In 56% the greatest diameter was > 60mm. There were 77 % R0 resections. Median Follow up was 1.8 years (0-8.8). 5 Year overall survival was 25,8 %. Median survival was 2.8 years. Survival was significantly influenced by R0 resection (R0 vs. R+, p=0.006), Nodal status (N0 vs. N+, P<0.001), number of tumors (=/> 1, p<0.001), T stage (T1/2 vs. 2/3, p=0.001) and presence of metastasis (M+ vs M0, p <0.001). Even in nodal negative and early T stages 5 year survival is below 35% (N0 = 32%, T1+2 = 31%). Tumor size (</> 60mm), gender (m/f) did not influence survival. Multivariate analysis showed significantly worse survival for nodal positive tumors (p<0.02, HR 2.7 (CI 1.2-6.2)), distant metastases (p<0.22, HR 3.3 (CI 1.2-9.1)) and T stage >2 (p<0.16 HR 2.4 (CI 1.2-4.8))

Conclusion: Intrahepatic cholangiocarcinomas (ICC) are a rare but aggressive entity with poor 5 year survival. Nodal negative R0 resections of single tumors with a low T stage and low grading have the best prognosis, whereas multicentric and nodal positive tumors seem to show poor survival despite surgical resection.In the light of poorly defined adjuvant therapies, studies like e.g. the ACTICCA study are very much needed in order to know which patients will benefit from these therapies. An attempt to surgically resect those tumors should be made whenever possible since e.g. nodal status , T stage and grading cannot reliably be determined preoperatively in all cases.

Figure 1 [Fig. 1]