Abstract
Intrahepatic cholangiocarcinoma (ICC) is a rare disease with poor outcome, despite advances in surgical and non-surgical treatment. Recently, studies have reported a favorable long-term outcome of “very early” ICC (based on tumor size and absence of extrahepatic disease) after hepatic resection and liver transplantation, respectively. However, the prognostic value of tumor size and a reliable definition of early disease remain a matter of debate. Patients undergoing resection of histologically confirmed ICC between February 1996 and January 2021 at our institution were reviewed for postoperative morbidity, mortality, and long-term outcome after being retrospectively assigned to two groups: “very early” (single tumor ≤ 3 cm) and “advanced” ICC (size > 3 cm, multifocality or extrahepatic disease). A total of 297 patients were included, with a median follow-up of 22.8 (0.1–301.7) months. Twenty-one (7.1%) patients underwent resection of “very early” ICC. Despite the small tumor size, major hepatectomies (defined as resection of ≥3 segments) were performed in 14 (66.7%) cases. Histopathological analyses revealed lymph node metastases in 5 (23.8%) patients. Patients displayed excellent postoperative outcome compared to patients with “advanced” disease: intrahospital mortality was not observed, and patients displayed superior long-term survival, with a 5-year survival rate of 58.2% (versus 24.3%) and a median postoperative survival of 62.1 months (versus 25.3 months; p = 0.013). In conclusion, although the concept of a “very early” ICC based solely on tumor size is vague as it does not necessarily reflect an aggressive tumor biology, our proposed definition could serve as a basis for further studies evaluating the efficiency of either surgical resection or liver transplantation for this malignant disease.
Highlights
Intrahepatic cholangiocarcinoma (ICC) is a highly malignant disease with poor prognosis and the second most common primary hepatic malignancy, responsible for 10–20% of all primary liver tumors [1,2].Recent epidemiological data revealed a rising incidence; ICC remains a rarity, especially compared to hepatocellular carcinoma [2]
There is a lack of large clinical studies to identify patients, which benefit most from surgical resections, several prognostic factors for postoperative survival have been identified in the past, including multifocality, vascular invasion, lymph node involvement, and type of liver resection [3,4,5,6,7]
The tumor diameter itself as a prognostic factor has been a matter of debate: while the first staging system of the American Joint Committee on Cancer (AJCC) did not include tumor size, the 8th edition of the AJCC(/UICC) defines stage IA and IB depending on a maximum tumor size of 5 cm [8,9]
Summary
Intrahepatic cholangiocarcinoma (ICC) is a highly malignant disease with poor prognosis and the second most common primary hepatic malignancy, responsible for 10–20% of all primary liver tumors [1,2].Recent epidemiological data revealed a rising incidence; ICC remains a rarity, especially compared to hepatocellular carcinoma [2]. The Liver Cancer Study Group of Japan demonstrated that a tumor cut-off diameter of 2 cm had a significant prognostic value in 419 patients undergoing resection of ICC [10]. Several reports were published illustrating the excellent long-term outcome of patients with a so-called “very early” ICC (single tumor ≤ 2 cm, absence of extrahepatic disease) undergoing liver transplantation for advanced cirrhosis and hepatic failure [11,12]. Data of patients with “very early” ICC undergoing surgical resection are scarce, especially as patients often present themselves with more advanced disease. Molina et al identified seven patients meeting the above-mentioned criteria for “very early” ICC in preoperative imaging in their study of 89 patients undergoing liver resection with an improved 5-year survival rate of 68.6% but failed to show statistically significant superior outcome [13]
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